Desmoid tumor differential diagnosis: Difference between revisions

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{{CMG}} {{AE}}{{S.M.}}
{{CMG}} {{AE}}{{S.M.}}
==Overview==
==Overview==
Extra-[[abdominal]] [[desmoid tumor]] must be differentiated from [[fibrosarcoma]], low-grade fibromyxoid [[sarcoma]] and Gardner [[fibroma]]. Intra-[[abdominal]] [[desmoid tumor]] must be differentiated from [[Gastrointestinal stromal tumor|gastrointestinal stromal tumor (GIST)]], [[benign]] [[Fibrous tumor of the pleura|fibrous tumor]]/[[Solitary fibrous tumor of the pleura|solitary fibrous tumor]] (SFT), [[inflammatory]] myofibroblastic [[tumor]] (IMT), sclerosing mesenteritis and [[retroperitoneal fibrosis]]. Furthermore, generally all [[Desmoid tumor|desmoid tumors]] must be differentiated from acute [[hematoma]], [[lymphoma]], [[rhabdomyosarcoma]], [[liposarcoma]], [[leiomyosarcoma]], [[neurofibroma]], [[nodular fasciitis]], [[Hypertrophic scars|hypertrophic scars,]][[keloids]] and [[primitive neuroectodermal tumor]].
Extra-[[abdominal]] [[desmoid tumor]] must be differentiated from [[fibrosarcoma]], low-grade fibromyxoid [[sarcoma]] and Gardner [[fibroma]]. Intra-[[abdominal]] [[desmoid tumor]] must be differentiated from [[Gastrointestinal stromal tumor|gastrointestinal stromal tumor (GIST)]], [[benign]] [[Fibrous tumor of the pleura|fibrous tumor]]/[[Solitary fibrous tumor of the pleura|solitary fibrous tumor]] (SFT), [[inflammatory]] myofibroblastic [[tumor]] (IMT), sclerosing mesenteritis and [[retroperitoneal fibrosis]]. Furthermore, generally all [[Desmoid tumor|desmoid tumors]] must be differentiated from [[Acute (medicine)|acute]] [[hematoma]], [[lymphoma]], [[rhabdomyosarcoma]], [[liposarcoma]], [[leiomyosarcoma]], [[neurofibroma]], [[nodular fasciitis]], [[Hypertrophic scars|hypertrophic scars,]][[keloids]] and [[primitive neuroectodermal tumor]].


==Differentiating Desmoid tumor from other Diseases==
==Differentiating Desmoid tumor from other Diseases==
*'''Extra-[[abdominal]] [[fibromatosis]]/[[desmoid tumor]]''' must be differentiated from:<ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref><ref name="pmid21478276">{{cite journal| author=Kasper B, Ströbel P, Hohenberger P| title=Desmoid tumors: clinical features and treatment options for advanced disease. | journal=Oncologist | year= 2011 | volume= 16 | issue= 5 | pages= 682-93 | pmid=21478276 | doi=10.1634/theoncologist.2010-0281 | pmc=3228186 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21478276  }} </ref><ref name="pmid17711447">{{cite journal| author=Carlson JW, Fletcher CD| title=Immunohistochemistry for beta-catenin in the differential diagnosis of spindle cell lesions: analysis of a series and review of the literature. | journal=Histopathology | year= 2007 | volume= 51 | issue= 4 | pages= 509-14 | pmid=17711447 | doi=10.1111/j.1365-2559.2007.02794.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17711447  }} </ref>
*'''Extra-[[abdominal]] [[fibromatosis]]/[[desmoid tumor]]''' must be differentiated from:<ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref><ref name="pmid21478276">{{cite journal| author=Kasper B, Ströbel P, Hohenberger P| title=Desmoid tumors: clinical features and treatment options for advanced disease. | journal=Oncologist | year= 2011 | volume= 16 | issue= 5 | pages= 682-93 | pmid=21478276 | doi=10.1634/theoncologist.2010-0281 | pmc=3228186 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21478276  }} </ref><ref name="pmid17711447">{{cite journal| author=Carlson JW, Fletcher CD| title=Immunohistochemistry for beta-catenin in the differential diagnosis of spindle cell lesions: analysis of a series and review of the literature. | journal=Histopathology | year= 2007 | volume= 51 | issue= 4 | pages= 509-14 | pmid=17711447 | doi=10.1111/j.1365-2559.2007.02794.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17711447 }} </ref><ref name="pmid19152188">{{cite journal| author=Wu X, Petrovic V, Torode IP, Chow CW| title=Low grade fibromyxoid sarcoma: problems in the diagnosis and management of a malignant tumour with bland histological appearance. | journal=Pathology | year= 2009 | volume= 41 | issue= 2 | pages= 155-60 | pmid=19152188 | doi=10.1080/00313020802579276 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19152188  }} </ref><ref name="pmid20471519">{{cite journal| author=Bartuma H, Möller E, Collin A, Domanski HA, Von Steyern FV, Mandahl N et al.| title=Fusion of the FUS and CREB3L2 genes in a supernumerary ring chromosome in low-grade fibromyxoid sarcoma. | journal=Cancer Genet Cytogenet | year= 2010 | volume= 199 | issue= 2 | pages= 143-6 | pmid=20471519 | doi=10.1016/j.cancergencyto.2010.02.011 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20471519  }} </ref><ref name="pmid11342777">{{cite journal| author=Wehrli BM, Weiss SW, Yandow S, Coffin CM| title=Gardner-associated fibromas (GAF) in young patients: a distinct fibrous lesion that identifies unsuspected Gardner syndrome and risk for fibromatosis. | journal=Am J Surg Pathol | year= 2001 | volume= 25 | issue= 5 | pages= 645-51 | pmid=11342777 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11342777 }} </ref>
**[[Fibrosarcoma]]/[[fibroblastic sarcoma]]
**[[Fibrosarcoma]]/[[fibroblastic sarcoma]]
**Low-grade fibromyxoid [[sarcoma]]<ref name="pmid19152188">{{cite journal| author=Wu X, Petrovic V, Torode IP, Chow CW| title=Low grade fibromyxoid sarcoma: problems in the diagnosis and management of a malignant tumour with bland histological appearance. | journal=Pathology | year= 2009 | volume= 41 | issue= 2 | pages= 155-60 | pmid=19152188 | doi=10.1080/00313020802579276 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19152188  }} </ref><ref name="pmid20471519">{{cite journal| author=Bartuma H, Möller E, Collin A, Domanski HA, Von Steyern FV, Mandahl N et al.| title=Fusion of the FUS and CREB3L2 genes in a supernumerary ring chromosome in low-grade fibromyxoid sarcoma. | journal=Cancer Genet Cytogenet | year= 2010 | volume= 199 | issue= 2 | pages= 143-6 | pmid=20471519 | doi=10.1016/j.cancergencyto.2010.02.011 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20471519  }} </ref>
**Low-grade fibromyxoid [[sarcoma]]
**Gardner [[fibroma]]<ref name="pmid11342777">{{cite journal| author=Wehrli BM, Weiss SW, Yandow S, Coffin CM| title=Gardner-associated fibromas (GAF) in young patients: a distinct fibrous lesion that identifies unsuspected Gardner syndrome and risk for fibromatosis. | journal=Am J Surg Pathol | year= 2001 | volume= 25 | issue= 5 | pages= 645-51 | pmid=11342777 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11342777  }} </ref>
**Gardner [[fibroma]]
*'''Intra-[[abdominal]] [[fibromatosis]]''' must be differentiated from:<ref name="pmid17414097">{{cite journal| author=Coffin CM, Hornick JL, Fletcher CD| title=Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. | journal=Am J Surg Pathol | year= 2007 | volume= 31 | issue= 4 | pages= 509-20 | pmid=17414097 | doi=10.1097/01.pas.0000213393.57322.c7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17414097  }} </ref>
*'''Intra-[[abdominal]] [[fibromatosis]]''' must be differentiated from:<ref name="pmid17414097">{{cite journal| author=Coffin CM, Hornick JL, Fletcher CD| title=Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. | journal=Am J Surg Pathol | year= 2007 | volume= 31 | issue= 4 | pages= 509-20 | pmid=17414097 | doi=10.1097/01.pas.0000213393.57322.c7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17414097  }} </ref>
**[[Gastrointestinal stromal tumor]] ([[GIST]])
**[[Gastrointestinal stromal tumor]] ([[GIST]])
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***Underlying [[malignancy]] such as [[lymphoma]]
***Underlying [[malignancy]] such as [[lymphoma]]
*Furthermore, generally all [[Desmoid tumor|desmoid tumors]]<nowiki/> must be differentiated from:
*Furthermore, generally all [[Desmoid tumor|desmoid tumors]]<nowiki/> must be differentiated from:
**Acute [[hematoma]]
**[[Acute (medicine)|Acute]] [[hematoma]]
**[[Lymphoma]]
**[[Lymphoma]]
**[[Rhabdomyosarcoma]]
**[[Rhabdomyosarcoma]]
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! style="background:#4479BA; color: #FFFFFF;" align="center" + |Other associated features
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Other associated features
|-
|-
|[[Desmoid tumor|'''Desmoid tumor''']]
| style="background:#DCDCDC;" align="center" + |[[Desmoid tumor|'''Desmoid tumor''']]
|Sporadic [[Desmoid tumor|desmoids]] are associated with following [[mutations]]:
|Sporadic [[Desmoid tumor|desmoids]] are associated with following [[mutations]]:
* [[Wnt signaling pathway|Wnt]]/[[beta-catenin]] [[signaling pathway]]
* [[Wnt signaling pathway|Wnt]]/[[beta-catenin]] [[signaling pathway]]
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* [[Cytokeratin|Cytokeratins]]
* [[Cytokeratin|Cytokeratins]]
* [[S-100]]
* [[S-100]]
* [[c-KIT]]
* [[c-KIT]]
|
|
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* [[Trisomy]] 20
* [[Trisomy]] 20
|-
|-
|'''[[Fibrosarcoma]]/[[Fibroblastic sarcoma]]'''
| style="background:#DCDCDC;" align="center" + |'''[[Fibrosarcoma]]/[[Fibroblastic sarcoma]]'''
|
|
* Unknown precise [[Causes|cause]]
* Unknown precise [[Causes|cause]]
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* [[CD31]]
* [[CD31]]
* [[CD34]]
* [[CD34]]
* [[Cytokeratin]]
* [[Cytokeratin]]
* [[Epithelial]] [[membrane]] [[antigen]]
* [[Epithelial]] [[membrane]] [[antigen]]
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* Moth-eaten [[appearance]] on [[X-ray]]
* Moth-eaten [[appearance]] on [[X-ray]]
|-
|-
|'''Low-grade fibromyxoid [[sarcoma]]'''<ref name="pmid19152188" /><ref name="pmid20471519" /><ref name="pmid21415703">{{cite journal| author=Doyle LA, Möller E, Dal Cin P, Fletcher CD, Mertens F, Hornick JL| title=MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma. | journal=Am J Surg Pathol | year= 2011 | volume= 35 | issue= 5 | pages= 733-41 | pmid=21415703 | doi=10.1097/PAS.0b013e318210c268 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21415703  }} </ref><ref name="pmid21658743">{{cite journal| author=Lee AF, Yip S, Smith AC, Hayes MM, Nielsen TO, O'Connell JX| title=Low-grade fibromyxoid sarcoma of the perineum with heterotopic ossification: case report and review of the literature. | journal=Hum Pathol | year= 2011 | volume= 42 | issue= 11 | pages= 1804-9 | pmid=21658743 | doi=10.1016/j.humpath.2011.01.023 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21658743  }} </ref><ref name="pmid22136982">{{cite journal| author=Brasanac D, Dzelatovic NS, Stojanovic M| title=Giant cystic superficial low-grade fibromyxoid sarcoma. | journal=Ann Diagn Pathol | year= 2013 | volume= 17 | issue= 2 | pages= 222-5 | pmid=22136982 | doi=10.1016/j.anndiagpath.2011.09.001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22136982  }} </ref><ref name="pmid16948525">{{cite journal| author=Vernon SE, Bejarano PA| title=Low-grade fibromyxoid sarcoma: a brief review. | journal=Arch Pathol Lab Med | year= 2006 | volume= 130 | issue= 9 | pages= 1358-60 | pmid=16948525 | doi=10.1043/1543-2165(2006)130[1358:LFSABR]2.0.CO;2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16948525  }} </ref><ref name="pmid9414192">{{cite journal| author=Lane KL, Shannon RJ, Weiss SW| title=Hyalinizing spindle cell tumor with giant rosettes: a distinctive tumor closely resembling low-grade fibromyxoid sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 12 | pages= 1481-8 | pmid=9414192 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9414192  }} </ref><ref name="pmid10524523">{{cite journal| author=Nielsen GP, Selig MK, O'Connell JX, Keel SB, Dickersin GR, Rosenberg AE| title=Hyalinizing spindle cell tumor with giant rosettes: a report of three cases with ultrastructural analysis. | journal=Am J Surg Pathol | year= 1999 | volume= 23 | issue= 10 | pages= 1227-32 | pmid=10524523 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10524523  }} </ref>
| style="background:#DCDCDC;" align="center" + |'''Low-grade fibromyxoid [[sarcoma]]'''<ref name="pmid19152188" /><ref name="pmid20471519" /><ref name="pmid21415703">{{cite journal| author=Doyle LA, Möller E, Dal Cin P, Fletcher CD, Mertens F, Hornick JL| title=MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma. | journal=Am J Surg Pathol | year= 2011 | volume= 35 | issue= 5 | pages= 733-41 | pmid=21415703 | doi=10.1097/PAS.0b013e318210c268 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21415703  }} </ref><ref name="pmid21658743">{{cite journal| author=Lee AF, Yip S, Smith AC, Hayes MM, Nielsen TO, O'Connell JX| title=Low-grade fibromyxoid sarcoma of the perineum with heterotopic ossification: case report and review of the literature. | journal=Hum Pathol | year= 2011 | volume= 42 | issue= 11 | pages= 1804-9 | pmid=21658743 | doi=10.1016/j.humpath.2011.01.023 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21658743  }} </ref><ref name="pmid22136982">{{cite journal| author=Brasanac D, Dzelatovic NS, Stojanovic M| title=Giant cystic superficial low-grade fibromyxoid sarcoma. | journal=Ann Diagn Pathol | year= 2013 | volume= 17 | issue= 2 | pages= 222-5 | pmid=22136982 | doi=10.1016/j.anndiagpath.2011.09.001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22136982  }} </ref><ref name="pmid16948525">{{cite journal| author=Vernon SE, Bejarano PA| title=Low-grade fibromyxoid sarcoma: a brief review. | journal=Arch Pathol Lab Med | year= 2006 | volume= 130 | issue= 9 | pages= 1358-60 | pmid=16948525 | doi=10.1043/1543-2165(2006)130[1358:LFSABR]2.0.CO;2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16948525  }} </ref><ref name="pmid9414192">{{cite journal| author=Lane KL, Shannon RJ, Weiss SW| title=Hyalinizing spindle cell tumor with giant rosettes: a distinctive tumor closely resembling low-grade fibromyxoid sarcoma. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 12 | pages= 1481-8 | pmid=9414192 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9414192  }} </ref><ref name="pmid10524523">{{cite journal| author=Nielsen GP, Selig MK, O'Connell JX, Keel SB, Dickersin GR, Rosenberg AE| title=Hyalinizing spindle cell tumor with giant rosettes: a report of three cases with ultrastructural analysis. | journal=Am J Surg Pathol | year= 1999 | volume= 23 | issue= 10 | pages= 1227-32 | pmid=10524523 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10524523  }} </ref>
|[[Translocation]]:
|[[Translocation]]:
* t(7;18;16) or
* t(7;18;16) or
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|Positive for:
|Positive for:
* MUC4 (highly specific and sensitive)
* MUC4 (highly specific and sensitive)
* [[Vimentin]]  
* [[Vimentin]]  
Occasionally positive for:
Occasionally positive for:
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|_
|_
|-
|-
|'''[[Benign]] [[Fibrous tumor of the pleura|fibrous tumor]][[Solitary fibrous tumor of the pleura|/Solitary fibrous tumor (SFT)]]''' <ref name="pmid7705824">{{cite journal| author=Hanau CA, Miettinen M| title=Solitary fibrous tumor: histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites. | journal=Hum Pathol | year= 1995 | volume= 26 | issue= 4 | pages= 440-9 | pmid=7705824 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7705824  }} </ref><ref name="pmid10349983">{{cite journal| author=de Saint Aubain Somerhausen N, Rubin BP, Fletcher CD| title=Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis. | journal=Mod Pathol | year= 1999 | volume= 12 | issue= 5 | pages= 463-71 | pmid=10349983 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10349983  }} </ref><ref name="pmid19345055">{{cite journal| author=Cranshaw IM, Gikas PD, Fisher C, Thway K, Thomas JM, Hayes AJ| title=Clinical outcomes of extra-thoracic solitary fibrous tumours. | journal=Eur J Surg Oncol | year= 2009 | volume= 35 | issue= 9 | pages= 994-8 | pmid=19345055 | doi=10.1016/j.ejso.2009.02.015 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19345055  }} </ref><ref name="pmid2665534">{{cite journal| author=England DM, Hochholzer L, McCarthy MJ| title=Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases. | journal=Am J Surg Pathol | year= 1989 | volume= 13 | issue= 8 | pages= 640-58 | pmid=2665534 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2665534  }} </ref><ref name="pmid22575866">{{cite journal| author=Demicco EG, Park MS, Araujo DM, Fox PS, Bassett RL, Pollock RE et al.| title=Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model. | journal=Mod Pathol | year= 2012 | volume= 25 | issue= 9 | pages= 1298-306 | pmid=22575866 | doi=10.1038/modpathol.2012.83 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22575866  }} </ref><ref name="pmid24052313">{{cite journal| author=van Houdt WJ, Westerveld CM, Vrijenhoek JE, van Gorp J, van Coevorden F, Verhoef C et al.| title=Prognosis of solitary fibrous tumors: a multicenter study. | journal=Ann Surg Oncol | year= 2013 | volume= 20 | issue= 13 | pages= 4090-5 | pmid=24052313 | doi=10.1245/s10434-013-3242-9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24052313  }} </ref><ref name="pmid23313954">{{cite journal| author=Chmielecki J, Crago AM, Rosenberg M, O'Connor R, Walker SR, Ambrogio L et al.| title=Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors. | journal=Nat Genet | year= 2013 | volume= 45 | issue= 2 | pages= 131-2 | pmid=23313954 | doi=10.1038/ng.2522 | pmc=3984043 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23313954  }} </ref><ref name="pmid23313952">{{cite journal| author=Robinson DR, Wu YM, Kalyana-Sundaram S, Cao X, Lonigro RJ, Sung YS et al.| title=Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. | journal=Nat Genet | year= 2013 | volume= 45 | issue= 2 | pages= 180-5 | pmid=23313952 | doi=10.1038/ng.2509 | pmc=3654808 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23313952  }} </ref><ref name="pmid23575898">{{cite journal| author=Schweizer L, Koelsche C, Sahm F, Piro RM, Capper D, Reuss DE et al.| title=Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein. | journal=Acta Neuropathol | year= 2013 | volume= 125 | issue= 5 | pages= 651-8 | pmid=23575898 | doi=10.1007/s00401-013-1117-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23575898  }} </ref>
| style="background:#DCDCDC;" align="center" + |'''[[Benign]] [[Fibrous tumor of the pleura|fibrous tumor]][[Solitary fibrous tumor of the pleura|/Solitary fibrous tumor (SFT)]]''' <ref name="pmid7705824">{{cite journal| author=Hanau CA, Miettinen M| title=Solitary fibrous tumor: histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites. | journal=Hum Pathol | year= 1995 | volume= 26 | issue= 4 | pages= 440-9 | pmid=7705824 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7705824  }} </ref><ref name="pmid10349983">{{cite journal| author=de Saint Aubain Somerhausen N, Rubin BP, Fletcher CD| title=Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis. | journal=Mod Pathol | year= 1999 | volume= 12 | issue= 5 | pages= 463-71 | pmid=10349983 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10349983  }} </ref><ref name="pmid19345055">{{cite journal| author=Cranshaw IM, Gikas PD, Fisher C, Thway K, Thomas JM, Hayes AJ| title=Clinical outcomes of extra-thoracic solitary fibrous tumours. | journal=Eur J Surg Oncol | year= 2009 | volume= 35 | issue= 9 | pages= 994-8 | pmid=19345055 | doi=10.1016/j.ejso.2009.02.015 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19345055  }} </ref><ref name="pmid2665534">{{cite journal| author=England DM, Hochholzer L, McCarthy MJ| title=Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases. | journal=Am J Surg Pathol | year= 1989 | volume= 13 | issue= 8 | pages= 640-58 | pmid=2665534 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2665534  }} </ref><ref name="pmid22575866">{{cite journal| author=Demicco EG, Park MS, Araujo DM, Fox PS, Bassett RL, Pollock RE et al.| title=Solitary fibrous tumor: a clinicopathological study of 110 cases and proposed risk assessment model. | journal=Mod Pathol | year= 2012 | volume= 25 | issue= 9 | pages= 1298-306 | pmid=22575866 | doi=10.1038/modpathol.2012.83 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22575866  }} </ref><ref name="pmid24052313">{{cite journal| author=van Houdt WJ, Westerveld CM, Vrijenhoek JE, van Gorp J, van Coevorden F, Verhoef C et al.| title=Prognosis of solitary fibrous tumors: a multicenter study. | journal=Ann Surg Oncol | year= 2013 | volume= 20 | issue= 13 | pages= 4090-5 | pmid=24052313 | doi=10.1245/s10434-013-3242-9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24052313  }} </ref><ref name="pmid23313954">{{cite journal| author=Chmielecki J, Crago AM, Rosenberg M, O'Connor R, Walker SR, Ambrogio L et al.| title=Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors. | journal=Nat Genet | year= 2013 | volume= 45 | issue= 2 | pages= 131-2 | pmid=23313954 | doi=10.1038/ng.2522 | pmc=3984043 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23313954  }} </ref><ref name="pmid23313952">{{cite journal| author=Robinson DR, Wu YM, Kalyana-Sundaram S, Cao X, Lonigro RJ, Sung YS et al.| title=Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. | journal=Nat Genet | year= 2013 | volume= 45 | issue= 2 | pages= 180-5 | pmid=23313952 | doi=10.1038/ng.2509 | pmc=3654808 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23313952  }} </ref><ref name="pmid23575898">{{cite journal| author=Schweizer L, Koelsche C, Sahm F, Piro RM, Capper D, Reuss DE et al.| title=Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein. | journal=Acta Neuropathol | year= 2013 | volume= 125 | issue= 5 | pages= 651-8 | pmid=23575898 | doi=10.1007/s00401-013-1117-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23575898  }} </ref>
|Intra-[[Chromosomal abnormality|chromosomal]] [[Inversion (kinesiology)|inversion]] at:
|Intra-[[Chromosomal abnormality|chromosomal]] [[Inversion (kinesiology)|inversion]] at:
* [[Chromosome]] 12q13 leading to [[Formation matrix|formation]] of ''[[NAB2]]–[[STAT6]]'' [[Chimeric protein|chimeric]] [[Fusion protein|fusion]] [[gene]] (highly variable breakpoints)
* [[Chromosome]] 12q13 leading to [[Formation matrix|formation]] of ''[[NAB2]]–[[STAT6]]'' [[Chimeric protein|chimeric]] [[Fusion protein|fusion]] [[gene]] (highly variable breakpoints)
Line 315: Line 312:
* [[Hypertrophic pulmonary osteoarthropathy|Hypertrophic pulmonary osteoarthropathy (HPO)]]/Pierre-Marie-Bamberger [[syndrome]]( in [[Pleuropulmonary blastoma|pleuropulmonary]] SFTs, secretes [[hyaluronic acid]], [[cytokines]])
* [[Hypertrophic pulmonary osteoarthropathy|Hypertrophic pulmonary osteoarthropathy (HPO)]]/Pierre-Marie-Bamberger [[syndrome]]( in [[Pleuropulmonary blastoma|pleuropulmonary]] SFTs, secretes [[hyaluronic acid]], [[cytokines]])
|-
|-
|'''Gardner fibroma/Gardner-associated fibroma (GAF)'''<ref name="CoffinHornick2007">{{cite journal|last1=Coffin|first1=Cheryl M.|last2=Hornick|first2=Jason L.|last3=Zhou|first3=Holly|last4=Fletcher|first4=Christopher D.M.|title=Gardner Fibroma: A Clinicopathologic and Immunohistochemical Analysis of 45 Patients With 57 Fibromas|journal=The American Journal of Surgical Pathology|volume=31|issue=3|year=2007|pages=410–416|issn=0147-5185|doi=10.1097/01.pas.0000213348.65014.0a}}</ref><ref name="DahlSheil2016">{{cite journal|last1=Dahl|first1=Nathan A.|last2=Sheil|first2=Amy|last3=Knapke|first3=Sarah|last4=Geller|first4=James I.|title=Gardner Fibroma|journal=Journal of Pediatric Hematology/Oncology|volume=38|issue=5|year=2016|pages=e154–e157|issn=1077-4114|doi=10.1097/MPH.0000000000000493}}</ref><ref name="pmid11342777">{{cite journal| author=Wehrli BM, Weiss SW, Yandow S, Coffin CM| title=Gardner-associated fibromas (GAF) in young patients: a distinct fibrous lesion that identifies unsuspected Gardner syndrome and risk for fibromatosis. | journal=Am J Surg Pathol | year= 2001 | volume= 25 | issue= 5 | pages= 645-51 | pmid=11342777 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11342777  }} </ref><ref name="pmid28018803">{{cite journal| author=Schäfer M, Kadmon M, Schmidt W, Treiber I, Moog U, Sutter C et al.| title=Neonatal Gardner Fibroma Leads to Detection of Familial Adenomatous Polyposis: Two Case Reports. | journal=European J Pediatr Surg Rep | year= 2016 | volume= 4 | issue= 1 | pages= 17-21 | pmid=28018803 | doi=10.1055/s-0036-1582443 | pmc=5177561 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28018803  }} </ref>
| style="background:#DCDCDC;" align="center" + |'''Gardner fibroma/Gardner-associated fibroma (GAF)'''<ref name="CoffinHornick2007">{{cite journal|last1=Coffin|first1=Cheryl M.|last2=Hornick|first2=Jason L.|last3=Zhou|first3=Holly|last4=Fletcher|first4=Christopher D.M.|title=Gardner Fibroma: A Clinicopathologic and Immunohistochemical Analysis of 45 Patients With 57 Fibromas|journal=The American Journal of Surgical Pathology|volume=31|issue=3|year=2007|pages=410–416|issn=0147-5185|doi=10.1097/01.pas.0000213348.65014.0a}}</ref><ref name="DahlSheil2016">{{cite journal|last1=Dahl|first1=Nathan A.|last2=Sheil|first2=Amy|last3=Knapke|first3=Sarah|last4=Geller|first4=James I.|title=Gardner Fibroma|journal=Journal of Pediatric Hematology/Oncology|volume=38|issue=5|year=2016|pages=e154–e157|issn=1077-4114|doi=10.1097/MPH.0000000000000493}}</ref><ref name="pmid11342777">{{cite journal| author=Wehrli BM, Weiss SW, Yandow S, Coffin CM| title=Gardner-associated fibromas (GAF) in young patients: a distinct fibrous lesion that identifies unsuspected Gardner syndrome and risk for fibromatosis. | journal=Am J Surg Pathol | year= 2001 | volume= 25 | issue= 5 | pages= 645-51 | pmid=11342777 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11342777  }} </ref><ref name="pmid28018803">{{cite journal| author=Schäfer M, Kadmon M, Schmidt W, Treiber I, Moog U, Sutter C et al.| title=Neonatal Gardner Fibroma Leads to Detection of Familial Adenomatous Polyposis: Two Case Reports. | journal=European J Pediatr Surg Rep | year= 2016 | volume= 4 | issue= 1 | pages= 17-21 | pmid=28018803 | doi=10.1055/s-0036-1582443 | pmc=5177561 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28018803  }} </ref>


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Line 352: Line 349:
* [[Precursor]] [[lesion]] for [[Colon|colonic]] [[adenomas]] in [[asymptomatic]] [[FAP]] [[patients]]
* [[Precursor]] [[lesion]] for [[Colon|colonic]] [[adenomas]] in [[asymptomatic]] [[FAP]] [[patients]]
|-
|-
|'''[[Gastrointestinal stromal tumor]] ([[GIST]])'''<ref name="pmid26055339">{{cite journal| author=Zarkavelis G, Petrakis D, Pavlidis N| title=Gastrointestinal stromal tumors during pregnancy: a systematic review of an uncommon but treatable malignancy. | journal=Clin Transl Oncol | year= 2015 | volume= 17 | issue= 10 | pages= 757-62 | pmid=26055339 | doi=10.1007/s12094-015-1315-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26055339  }} </ref><ref name="pmid27384431">{{cite journal| author=Navarrete A, Momblán D, Almenara R, Lacy A| title=Giant Gastric Gastrointestinal Stromal Tumor (GIST). | journal=J Gastrointest Surg | year= 2017 | volume= 21 | issue= 1 | pages= 202-204 | pmid=27384431 | doi=10.1007/s11605-016-3196-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27384431  }} </ref><ref name="pmid19763238">{{cite journal| author=Scherjon S, Lam WF, Gelderblom H, Jansen FW| title=Gastrointestinal stromal tumor in pregnancy: a case report. | journal=Case Rep Med | year= 2009 | volume= 2009 | issue=  | pages= 456402 | pmid=19763238 | doi=10.1155/2009/456402 | pmc=2745024 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19763238  }} </ref><ref name="pmid22472152">{{cite journal| author=Acín-Gándara D, Pereira-Pérez F, Castaño-Pascual A, Durán-Poveda M, Antequera-Pérez A, Miliani-Molina C| title=Gastrointestinal stromal tumors: diagnosis and treatment. | journal=Cir Cir | year= 2012 | volume= 80 | issue= 1 | pages= 44-51 | pmid=22472152 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22472152  }} </ref><ref name="pmid16413690">{{cite journal| author=Lanzafame S, Minutolo V, Caltabiano R, Minutolo O, Marino B, Gagliano G et al.| title=About a case of GIST occurring during pregnancy with immunohistochemical expression of epidermal growth factor receptor and progesterone receptor. | journal=Pathol Res Pract | year= 2006 | volume= 202 | issue= 2 | pages= 119-23 | pmid=16413690 | doi=10.1016/j.prp.2005.08.013 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16413690  }} </ref>
| style="background:#DCDCDC;" align="center" + |'''[[Gastrointestinal stromal tumor]] ([[GIST]])'''<ref name="pmid26055339">{{cite journal| author=Zarkavelis G, Petrakis D, Pavlidis N| title=Gastrointestinal stromal tumors during pregnancy: a systematic review of an uncommon but treatable malignancy. | journal=Clin Transl Oncol | year= 2015 | volume= 17 | issue= 10 | pages= 757-62 | pmid=26055339 | doi=10.1007/s12094-015-1315-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26055339  }} </ref><ref name="pmid27384431">{{cite journal| author=Navarrete A, Momblán D, Almenara R, Lacy A| title=Giant Gastric Gastrointestinal Stromal Tumor (GIST). | journal=J Gastrointest Surg | year= 2017 | volume= 21 | issue= 1 | pages= 202-204 | pmid=27384431 | doi=10.1007/s11605-016-3196-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27384431  }} </ref><ref name="pmid19763238">{{cite journal| author=Scherjon S, Lam WF, Gelderblom H, Jansen FW| title=Gastrointestinal stromal tumor in pregnancy: a case report. | journal=Case Rep Med | year= 2009 | volume= 2009 | issue=  | pages= 456402 | pmid=19763238 | doi=10.1155/2009/456402 | pmc=2745024 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19763238  }} </ref><ref name="pmid22472152">{{cite journal| author=Acín-Gándara D, Pereira-Pérez F, Castaño-Pascual A, Durán-Poveda M, Antequera-Pérez A, Miliani-Molina C| title=Gastrointestinal stromal tumors: diagnosis and treatment. | journal=Cir Cir | year= 2012 | volume= 80 | issue= 1 | pages= 44-51 | pmid=22472152 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22472152  }} </ref><ref name="pmid16413690">{{cite journal| author=Lanzafame S, Minutolo V, Caltabiano R, Minutolo O, Marino B, Gagliano G et al.| title=About a case of GIST occurring during pregnancy with immunohistochemical expression of epidermal growth factor receptor and progesterone receptor. | journal=Pathol Res Pract | year= 2006 | volume= 202 | issue= 2 | pages= 119-23 | pmid=16413690 | doi=10.1016/j.prp.2005.08.013 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16413690  }} </ref>
|[[Activating group|Activating]] [[mutation]] of:
|[[Activating group|Activating]] [[mutation]] of:
* ''[[C-KIT]]'' ( [[tyrosine kinase|tyrosine kinase growth factor receptor]]) [[gene]] ([[Exon]] 9,13 & 17)
* ''[[C-KIT]]'' ( [[tyrosine kinase|tyrosine kinase growth factor receptor]]) [[gene]] ([[Exon]] 9,13 & 17)
Line 362: Line 359:
[[GIST]] as a part of [[Carney triad]] has following [[mutation]]:
[[GIST]] as a part of [[Carney triad]] has following [[mutation]]:
* Hypermethylation of the [[SDHC (gene)|SDHC]] [[promoter]] causing [[epigenetic]] inactivation of the [[SDHC (gene)|''SDHC'' gene]]  
* Hypermethylation of the [[SDHC (gene)|SDHC]] [[promoter]] causing [[epigenetic]] inactivation of the [[SDHC (gene)|''SDHC'' gene]]  
[[Wild-type]] GISTs have following three [[molecular]] subtypes:
[[Wild-type]] [[Gastrointestinal stromal tumor|GISTs]] have following three [[molecular]] subtypes:
* ''[[DHX15|DHX]]'' [[mutations]] (66%)
* ''[[DHX15|DHX]]'' [[mutations]] (66%)
* [[SDHC]] [[promoter]] hypermethylation (22%)
* [[SDHC]] [[promoter]] hypermethylation (22%)
Line 427: Line 424:
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|-
|'''[[Inflammatory]] myofibroblastic [[tumor]] (IMT)'''<ref name="pmid7611533">{{cite journal| author=Coffin CM, Watterson J, Priest JR, Dehner LP| title=Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. | journal=Am J Surg Pathol | year= 1995 | volume= 19 | issue= 8 | pages= 859-72 | pmid=7611533 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7611533  }} </ref><ref name="pmid8635024">{{cite journal| author=Wenig BM, Devaney K, Bisceglia M| title=Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. | journal=Cancer | year= 1995 | volume= 76 | issue= 11 | pages= 2217-29 | pmid=8635024 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8635024  }} </ref><ref name="pmid8847061">{{cite journal| author=Ramachandra S, Hollowood K, Bisceglia M, Fletcher CD| title=Inflammatory pseudotumour of soft tissues: a clinicopathological and immunohistochemical analysis of 18 cases. | journal=Histopathology | year= 1995 | volume= 27 | issue= 4 | pages= 313-23 | pmid=8847061 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8847061  }} </ref><ref name="pmid12673560">{{cite journal| author=Häusler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B| title=Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review. | journal=Hum Pathol | year= 2003 | volume= 34 | issue= 3 | pages= 253-62 | pmid=12673560 | doi=10.1053/hupa.2003.35 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12673560  }} </ref><ref name="pmid16160478">{{cite journal| author=Rabban JT, Zaloudek CJ, Shekitka KM, Tavassoli FA| title=Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors. | journal=Am J Surg Pathol | year= 2005 | volume= 29 | issue= 10 | pages= 1348-55 | pmid=16160478 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16160478  }} </ref><ref name="pmid16967468">{{cite journal| author=Kovach SJ, Fischer AC, Katzman PJ, Salloum RM, Ettinghausen SE, Madeb R et al.| title=Inflammatory myofibroblastic tumors. | journal=J Surg Oncol | year= 2006 | volume= 94 | issue= 5 | pages= 385-91 | pmid=16967468 | doi=10.1002/jso.20516 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16967468  }} </ref><ref name="pmid9606802">{{cite journal| author=Coffin CM, Dehner LP, Meis-Kindblom JM| title=Inflammatory myofibroblastic tumor, inflammatory fibrosarcoma, and related lesions: an historical review with differential diagnostic considerations. | journal=Semin Diagn Pathol | year= 1998 | volume= 15 | issue= 2 | pages= 102-10 | pmid=9606802 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9606802  }} </ref><ref name="pmid6336632">{{cite journal| author=Berardi RS, Lee SS, Chen HP, Stines GJ| title=Inflammatory pseudotumors of the lung. | journal=Surg Gynecol Obstet | year= 1983 | volume= 156 | issue= 1 | pages= 89-96 | pmid=6336632 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6336632  }} </ref><ref name="pmid8635024">{{cite journal| author=Wenig BM, Devaney K, Bisceglia M| title=Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. | journal=Cancer | year= 1995 | volume= 76 | issue= 11 | pages= 2217-29 | pmid=8635024 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8635024  }} </ref><ref name="pmid16160478">{{cite journal| author=Rabban JT, Zaloudek CJ, Shekitka KM, Tavassoli FA| title=Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors. | journal=Am J Surg Pathol | year= 2005 | volume= 29 | issue= 10 | pages= 1348-55 | pmid=16160478 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16160478  }} </ref><ref name="pmid17414097">{{cite journal| author=Coffin CM, Hornick JL, Fletcher CD| title=Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. | journal=Am J Surg Pathol | year= 2007 | volume= 31 | issue= 4 | pages= 509-20 | pmid=17414097 | doi=10.1097/01.pas.0000213393.57322.c7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17414097  }} </ref>
| style="background:#DCDCDC;" align="center" + |'''[[Inflammatory]] myofibroblastic [[tumor]] (IMT)'''<ref name="pmid7611533">{{cite journal| author=Coffin CM, Watterson J, Priest JR, Dehner LP| title=Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. | journal=Am J Surg Pathol | year= 1995 | volume= 19 | issue= 8 | pages= 859-72 | pmid=7611533 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7611533  }} </ref><ref name="pmid8635024">{{cite journal| author=Wenig BM, Devaney K, Bisceglia M| title=Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. | journal=Cancer | year= 1995 | volume= 76 | issue= 11 | pages= 2217-29 | pmid=8635024 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8635024  }} </ref><ref name="pmid8847061">{{cite journal| author=Ramachandra S, Hollowood K, Bisceglia M, Fletcher CD| title=Inflammatory pseudotumour of soft tissues: a clinicopathological and immunohistochemical analysis of 18 cases. | journal=Histopathology | year= 1995 | volume= 27 | issue= 4 | pages= 313-23 | pmid=8847061 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8847061  }} </ref><ref name="pmid12673560">{{cite journal| author=Häusler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B| title=Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review. | journal=Hum Pathol | year= 2003 | volume= 34 | issue= 3 | pages= 253-62 | pmid=12673560 | doi=10.1053/hupa.2003.35 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12673560  }} </ref><ref name="pmid16160478">{{cite journal| author=Rabban JT, Zaloudek CJ, Shekitka KM, Tavassoli FA| title=Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors. | journal=Am J Surg Pathol | year= 2005 | volume= 29 | issue= 10 | pages= 1348-55 | pmid=16160478 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16160478  }} </ref><ref name="pmid16967468">{{cite journal| author=Kovach SJ, Fischer AC, Katzman PJ, Salloum RM, Ettinghausen SE, Madeb R et al.| title=Inflammatory myofibroblastic tumors. | journal=J Surg Oncol | year= 2006 | volume= 94 | issue= 5 | pages= 385-91 | pmid=16967468 | doi=10.1002/jso.20516 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16967468  }} </ref><ref name="pmid9606802">{{cite journal| author=Coffin CM, Dehner LP, Meis-Kindblom JM| title=Inflammatory myofibroblastic tumor, inflammatory fibrosarcoma, and related lesions: an historical review with differential diagnostic considerations. | journal=Semin Diagn Pathol | year= 1998 | volume= 15 | issue= 2 | pages= 102-10 | pmid=9606802 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9606802  }} </ref><ref name="pmid6336632">{{cite journal| author=Berardi RS, Lee SS, Chen HP, Stines GJ| title=Inflammatory pseudotumors of the lung. | journal=Surg Gynecol Obstet | year= 1983 | volume= 156 | issue= 1 | pages= 89-96 | pmid=6336632 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6336632  }} </ref><ref name="pmid8635024">{{cite journal| author=Wenig BM, Devaney K, Bisceglia M| title=Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. | journal=Cancer | year= 1995 | volume= 76 | issue= 11 | pages= 2217-29 | pmid=8635024 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8635024  }} </ref><ref name="pmid16160478">{{cite journal| author=Rabban JT, Zaloudek CJ, Shekitka KM, Tavassoli FA| title=Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors. | journal=Am J Surg Pathol | year= 2005 | volume= 29 | issue= 10 | pages= 1348-55 | pmid=16160478 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16160478  }} </ref><ref name="pmid17414097">{{cite journal| author=Coffin CM, Hornick JL, Fletcher CD| title=Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. | journal=Am J Surg Pathol | year= 2007 | volume= 31 | issue= 4 | pages= 509-20 | pmid=17414097 | doi=10.1097/01.pas.0000213393.57322.c7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17414097  }} </ref>
|Unknown underlying [[etiology]], may be due to [[inflammatory]] reaction to:
|Unknown underlying [[etiology]], may be due to [[inflammatory]] reaction to:
* [[Infection]]
* [[Infection]]
Line 437: Line 434:
* [[Spindle cells]] arranged in short [[fascicles]] with a focal storiform (whorled or cartwheel-like) architecture
* [[Spindle cells]] arranged in short [[fascicles]] with a focal storiform (whorled or cartwheel-like) architecture
* [[Spindle cells]] show features of [[fibroblasts]] and [[myofibroblasts]]
* [[Spindle cells]] show features of [[fibroblasts]] and [[myofibroblasts]]
* Variably dense, chronic, mixed [[polymorphic]] [[infiltrate]] of [[Mononuclear cells|mononuclear]] [[inflammatory]] ([[plasma cells]] and [[lymphocytes]], [[histiocytes]], [[neutrophils]], and occasional [[eosinophils]])
* Variably dense, chronic, mixed [[polymorphic]] [[infiltrate]] of [[Mononuclear cells|mononuclear]] [[inflammatory]] [[Cells (biology)|cells]] ([[plasma cells]] and [[lymphocytes]], [[histiocytes]], [[neutrophils]], and occasional [[eosinophils]])
* [[Histiocytes]] have [[multinucleated]] forms with finely vacuolated [[cytoplasmic]] lipid droplets
* [[Histiocytes]] have [[multinucleated]] forms with finely vacuolated [[cytoplasmic]] [[lipid]] droplets
* Plasma cells with cytoplasmic Russell bodies (globular cytoplasmic inclusions of immunoglobulin) and polyclonal pattern of light chain expression
* [[Plasma cells]] with [[cytoplasmic]] [[Russell bodies]] ([[Globular protein|globular]] [[cytoplasmic]] [[inclusions]] of [[immunoglobulin]]) and [[polyclonal]] pattern of [[light chain]] [[expression]]
* Absent hyperchromasia and atypical mitoses
* Absent hyperchromasia and atypical [[mitoses]]
|Positive for:
|Positive for:
*IG+ (plasma cells)
*IG+ ([[plasma cells]])
*IL-1
*[[IL-1]]
*IL-6
*[[Interleukin 6|IL-6]]
*Smooth muscle actin
*[[Smooth muscle]] [[actin]]
*Desmin
*[[Desmin]]
*Calponin  
*[[Calponin]]
*Activin-like kinase 1  
*[[Activin]]-like [[kinase]] 1  
Negative for:
Negative for:
* Beta-catenin  
* [[Beta-catenin]]
|
|
* Metastatic
* [[Metastatic]]
|
|
* Multiorgan disease in association with chronic persistent Eikenella corrodens infection
* [[Multiple organ dysfunction syndrome|Multiorgan disease]] in association with [[Chronic (medical)|chronic]] persistent [[Eikenella corrodens]] [[infection]]
* Epstein Barr virus infection
* [[Epstein-Barr Virus|Epstein Barr virus]] [[infection]]
* Human herpes virus (HHV)-8 infection (Kaposi's sarcoma, multicentric Castleman's disease)
* [[HHV-8|Human herpes virus-8(HHV-8)]] [[infection]] ([[Kaposi's sarcoma]], multicentric [[Castleman's disease]])
|
|
* Lungs
* [[Lungs]]
* Gastrointestinal system  
* [[Gastrointestinal system]]
* Pelvic region  
* [[Pelvic]] region  
**Bladder
**[[Urinary bladder|Bladder]]
**Uterus
**[[Uterus]]
* Retroperitoneum  
* [[Retroperitoneum]]
* Skin
* [[Skin]]
* Bone (femur, temporal bone, jaw bone)
* [[Bone]] ([[femur]], [[temporal bone]], [[jaw]] [[bone]])
* CNS
* [[CNS]]
* Soft tissues
* [[Soft tissue|Soft tissues]]
* Larynx
* [[Larynx]]
* Heart (right ventricle is most commonly involved)
* [[Heart]] ([[right ventricle]] is most commonly involved)
* Pancreas (rarely)
* [[Pancreas]] (rarely)
|
|
* Asymptomatic (70%)
* [[Asymptomatic]] (70%)
*Painless asymptomatic mass/lump/swelling
*Painless [[asymptomatic]] [[mass]]/[[lump]]/[[swelling]]
* Pulmonary IMT presents as:
* [[Pulmonary]] IMT presents as:
** Chest pain
** [[Chest pain]]
** Cough
** [[Cough]]
** Dyspnea
** [[Dyspnea]]
** Hemoptysis (recurrent)
** [[Hemoptysis]] (recurrent)
** Fever
** [[Fever]]
** Fatigue
** [[Fatigue]]
** Weight loss
** [[Weight loss]]
** Appetite loss
** [[Appetite loss]]
*Bone IMT presents with:
*[[Bone]] [[IMT]] presents with:
**Mild bone pain
**Mild [[bone pain]]
**Easy fractures
**Easy [[fractures]]
**Headache
**[[Headache]]
**Dizziness
**[[Dizziness]]
**Numbness at tumor site
**[[Numbness]] at [[tumor]] site
**Bone marrow involvement in some cases
**[[Bone marrow]] involvement in some cases
*Heart IMT presents with:
*[[Heart]] [[IMT]] presents with:
**Chest pain
**[[Chest pain]]
**Difficulty breathing
**[[Difficulty breathing]]
**Palpitations
**[[Palpitations]]
**Fainting
**[[Fainting]]
**Obstruction of blood flow in the heart (large tumors)
**[[Obstruction]] of [[blood flow]] in the [[heart]] (large [[tumors]])
*Bladder IMT presents with:
*[[Urinary bladder|Bladder]] [[IMT]] presents with:
**Painless hematuria
**Painless [[hematuria]]
**Chronic pelvic pain
**[[Chronic (medical)|Chronic]] [[pelvic pain]]
**Difficulty in urinating
**[[Difficulty passing urine|Difficulty in urinating]]
**Presence of burning sensation
**Presence of [[Burning sensation throughout the urethra|burning sensation]]
*CNS IMT presents with:
*[[CNS]] [[IMT]] presents with:
**Presence of solitary or multiple tumors at various locations in the brain
**Presence of [[solitary]] or multiple [[tumors]] at various [[Location parameter|location]]<nowiki/>s in the [[brain]]
**Recurrent headaches
**Recurrent [[headaches]]
**Headache
**[[Headache]]
**Nausea and vomiting
**[[Nausea and vomiting]]
**Blurred vision
**[[Blurred vision]]
**Double vision
**[[Double vision]]
**Drooping of the eyelid
**[[Ptosis|Drooping of the eyelid]]
**Dizziness
**[[Dizziness]]
**Back pain (if spine involved)
**[[Back pain]] (if [[spine]] involved)
**Seizures
**[[Seizures]]
|Also known as:
|Also known as:
* Pseudo-inflammatory tumors
* Pseudo-[[inflammatory]] [[tumors]]
* Inflammatory pseudotumor
* [[Inflammatory]] pseudotumor
* Plasma cell granuloma
* [[Plasma cell]] [[granuloma]]
* Inflammatory pseudotumor
* [[Inflammatory]] pseudotumor
* Fibrous histiocytoma
* [[Fibrous histiocytoma]]
* Fibroxanthoma
* [[Fibroxanthoma]]
* Xanthogranuloma
* [[Xanthogranuloma]]
* Inflammatory pseudosarcoma
* [[Inflammatory]] pseudosarcoma
*Atypical fibromyxoid tumor
*Atypical fibromyxoid [[tumor]]
*Atypical myfibroblastic tumor
*Atypical myfibroblastic [[tumor]]
|-
|-
|'''Sclerosing mesenteritis'''<ref name="pmid835091">{{cite journal| author=Durst AL, Freund H, Rosenmann E, Birnbaum D| title=Mesenteric panniculitis: review of the leterature and presentation of cases. | journal=Surgery | year= 1977 | volume= 81 | issue= 2 | pages= 203-11 | pmid=835091 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=835091  }} </ref><ref name="pmid4499963">{{cite journal| author=Kipfer RE, Moertel CG, Dahlin DC| title=Mesenteric lipodystrophy. | journal=Ann Intern Med | year= 1974 | volume= 80 | issue= 5 | pages= 582-8 | pmid=4499963 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4499963  }} </ref><ref name="pmid2660295">{{cite journal| author=Monahan DW, Poston WK, Brown GJ| title=Mesenteric panniculitis. | journal=South Med J | year= 1989 | volume= 82 | issue= 6 | pages= 782-4 | pmid=2660295 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2660295  }} </ref><ref name="pmid9697910">{{cite journal| author=Parra-Davila E, McKenney MG, Sleeman D, Hartmann R, Rao RK, McKenney K et al.| title=Mesenteric panniculitis: case report and literature review. | journal=Am Surg | year= 1998 | volume= 64 | issue= 8 | pages= 768-71 | pmid=9697910 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9697910  }} </ref><ref name="pmid2658633">{{cite journal| author=Kelly JK, Hwang WS| title=Idiopathic retractile (sclerosing) mesenteritis and its differential diagnosis. | journal=Am J Surg Pathol | year= 1989 | volume= 13 | issue= 6 | pages= 513-21 | pmid=2658633 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2658633  }} </ref><ref name="pmid9130985">{{cite journal| author=Emory TS, Monihan JM, Carr NJ, Sobin LH| title=Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity? | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 4 | pages= 392-8 | pmid=9130985 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9130985  }} </ref><ref name="pmid14295938">{{cite journal| author=OGDEN WW, BRADBURN DM, RIVES JD| title=MESENTERIC PANNICULITIS: REVIEW OF 27 CASES. | journal=Ann Surg | year= 1965 | volume= 161 | issue=  | pages= 864-75 | pmid=14295938 | doi= | pmc=1409094 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14295938  }} </ref><ref name="pmid3176478">{{cite journal| author=Khachaturian T, Hughes J| title=Mesenteric panniculitis. | journal=West J Med | year= 1988 | volume= 148 | issue= 6 | pages= 700-1 | pmid=3176478 | doi= | pmc=1026221 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3176478  }} </ref><ref name="pmid10658720">{{cite journal| author=Daskalogiannaki M, Voloudaki A, Prassopoulos P, Magkanas E, Stefanaki K, Apostolaki E et al.| title=CT evaluation of mesenteric panniculitis: prevalence and associated diseases. | journal=AJR Am J Roentgenol | year= 2000 | volume= 174 | issue= 2 | pages= 427-31 | pmid=10658720 | doi=10.2214/ajr.174.2.1740427 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10658720  }} </ref><ref name="pmid17478346">{{cite journal| author=Akram S, Pardi DS, Schaffner JA, Smyrk TC| title=Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients. | journal=Clin Gastroenterol Hepatol | year= 2007 | volume= 5 | issue= 5 | pages= 589-96; quiz 523-4 | pmid=17478346 | doi=10.1016/j.cgh.2007.02.032 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17478346  }} </ref><ref name="pmid28197781">{{cite journal| author=Sharma P, Yadav S, Needham CM, Feuerstadt P| title=Sclerosing mesenteritis: a systematic review of 192 cases. | journal=Clin J Gastroenterol | year= 2017 | volume= 10 | issue= 2 | pages= 103-111 | pmid=28197781 | doi=10.1007/s12328-017-0716-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28197781  }} </ref><ref name="pmid7552643">{{cite journal| author=van der Hulst RW, Rauws EA, Tytgat GN| title=Mesenteritis secondary to the use of a pneumatic jackhammer. | journal=Eur J Gastroenterol Hepatol | year= 1995 | volume= 7 | issue= 6 | pages= 573-5 | pmid=7552643 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7552643  }} </ref><ref name="pmid15622594">{{cite journal| author=Mathew J, McKenna F, Mason J, Haboubi NY, Borghol M| title=Sclerosing mesenteritis with occult ileal perforation: report of a case simulating extensive intra-abdominal malignancy. | journal=Dis Colon Rectum | year= 2004 | volume= 47 | issue= 11 | pages= 1974-7 | pmid=15622594 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15622594  }} </ref>
| style="background:#DCDCDC;" align="center" + |'''Sclerosing mesenteritis'''<ref name="pmid835091">{{cite journal| author=Durst AL, Freund H, Rosenmann E, Birnbaum D| title=Mesenteric panniculitis: review of the leterature and presentation of cases. | journal=Surgery | year= 1977 | volume= 81 | issue= 2 | pages= 203-11 | pmid=835091 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=835091  }} </ref><ref name="pmid4499963">{{cite journal| author=Kipfer RE, Moertel CG, Dahlin DC| title=Mesenteric lipodystrophy. | journal=Ann Intern Med | year= 1974 | volume= 80 | issue= 5 | pages= 582-8 | pmid=4499963 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4499963  }} </ref><ref name="pmid2660295">{{cite journal| author=Monahan DW, Poston WK, Brown GJ| title=Mesenteric panniculitis. | journal=South Med J | year= 1989 | volume= 82 | issue= 6 | pages= 782-4 | pmid=2660295 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2660295  }} </ref><ref name="pmid9697910">{{cite journal| author=Parra-Davila E, McKenney MG, Sleeman D, Hartmann R, Rao RK, McKenney K et al.| title=Mesenteric panniculitis: case report and literature review. | journal=Am Surg | year= 1998 | volume= 64 | issue= 8 | pages= 768-71 | pmid=9697910 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9697910  }} </ref><ref name="pmid2658633">{{cite journal| author=Kelly JK, Hwang WS| title=Idiopathic retractile (sclerosing) mesenteritis and its differential diagnosis. | journal=Am J Surg Pathol | year= 1989 | volume= 13 | issue= 6 | pages= 513-21 | pmid=2658633 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2658633  }} </ref><ref name="pmid9130985">{{cite journal| author=Emory TS, Monihan JM, Carr NJ, Sobin LH| title=Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity? | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 4 | pages= 392-8 | pmid=9130985 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9130985  }} </ref><ref name="pmid14295938">{{cite journal| author=OGDEN WW, BRADBURN DM, RIVES JD| title=MESENTERIC PANNICULITIS: REVIEW OF 27 CASES. | journal=Ann Surg | year= 1965 | volume= 161 | issue=  | pages= 864-75 | pmid=14295938 | doi= | pmc=1409094 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14295938  }} </ref><ref name="pmid3176478">{{cite journal| author=Khachaturian T, Hughes J| title=Mesenteric panniculitis. | journal=West J Med | year= 1988 | volume= 148 | issue= 6 | pages= 700-1 | pmid=3176478 | doi= | pmc=1026221 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3176478  }} </ref><ref name="pmid10658720">{{cite journal| author=Daskalogiannaki M, Voloudaki A, Prassopoulos P, Magkanas E, Stefanaki K, Apostolaki E et al.| title=CT evaluation of mesenteric panniculitis: prevalence and associated diseases. | journal=AJR Am J Roentgenol | year= 2000 | volume= 174 | issue= 2 | pages= 427-31 | pmid=10658720 | doi=10.2214/ajr.174.2.1740427 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10658720  }} </ref><ref name="pmid17478346">{{cite journal| author=Akram S, Pardi DS, Schaffner JA, Smyrk TC| title=Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients. | journal=Clin Gastroenterol Hepatol | year= 2007 | volume= 5 | issue= 5 | pages= 589-96; quiz 523-4 | pmid=17478346 | doi=10.1016/j.cgh.2007.02.032 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17478346  }} </ref><ref name="pmid28197781">{{cite journal| author=Sharma P, Yadav S, Needham CM, Feuerstadt P| title=Sclerosing mesenteritis: a systematic review of 192 cases. | journal=Clin J Gastroenterol | year= 2017 | volume= 10 | issue= 2 | pages= 103-111 | pmid=28197781 | doi=10.1007/s12328-017-0716-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28197781  }} </ref><ref name="pmid7552643">{{cite journal| author=van der Hulst RW, Rauws EA, Tytgat GN| title=Mesenteritis secondary to the use of a pneumatic jackhammer. | journal=Eur J Gastroenterol Hepatol | year= 1995 | volume= 7 | issue= 6 | pages= 573-5 | pmid=7552643 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7552643  }} </ref><ref name="pmid15622594">{{cite journal| author=Mathew J, McKenna F, Mason J, Haboubi NY, Borghol M| title=Sclerosing mesenteritis with occult ileal perforation: report of a case simulating extensive intra-abdominal malignancy. | journal=Dis Colon Rectum | year= 2004 | volume= 47 | issue= 11 | pages= 1974-7 | pmid=15622594 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15622594  }} </ref>


<ref name="pmid14615565">{{cite journal| author=Horton KM, Lawler LP, Fishman EK| title=CT findings in sclerosing mesenteritis (panniculitis): spectrum of disease. | journal=Radiographics | year= 2003 | volume= 23 | issue= 6 | pages= 1561-7 | pmid=14615565 | doi=10.1148/rg.1103035010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14615565  }} </ref><ref name="pmid10705180">{{cite journal| author=Papadaki HA, Kouroumalis EA, Stefanaki K, Roussomoustakaki M, Daskalogiannaki ME, Reppa D et al.| title=Retractile mesenteritis presenting as fever of unknown origin and autoimmune haemolytic anaemia. | journal=Digestion | year= 2000 | volume= 61 | issue= 2 | pages= 145-8 | pmid=10705180 | doi=10.1159/000007748 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10705180  }} </ref><ref name="pmid11818670">{{cite journal| author=Vidarsson B, Matthíasson P, Agnarsson BA, Onundarson PT| title=Mesenteric panniculitis presenting with autoimmune haemolytic anaemia. | journal=Acta Haematol | year= 2002 | volume= 107 | issue= 1 | pages= 35-7 | pmid=11818670 | doi=10.1159/000046627 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11818670  }} </ref><ref name="pmid8832610">{{cite journal| author=Vernace MA, Bellucci AG, Mossey RT, Susin M, Mailloux LU, Wilkes BM et al.| title=Minimal change nephropathy associated with sclerosing mesenteritis. | journal=Nephron | year= 1996 | volume= 73 | issue= 3 | pages= 473-6 | pmid=8832610 | doi=10.1159/000189113 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8832610  }} </ref><ref name="pmid7103574">{{cite journal| author=Dor AM, Kohler JL, Aubrespy P, Scheiner C, Pizzi M, Lebreuil G| title=[Mesenteric panniculitis, an unusual initial stage of acute lupus erythematosus in a ten-year old girl (author's transl)]. | journal=Arch Anat Cytol Pathol | year= 1982 | volume= 30 | issue= 2 | pages= 121-4 | pmid=7103574 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7103574  }} </ref><ref name="pmid8378657">{{cite journal| author=Sauvaget F, Piette JC, Galezowski N, Jouanique C, Chapelon C, Blétry O et al.| title=[Relapsing polychondritis and mesenteric panniculitis: apropos of 2 cases]. | journal=Rev Med Interne | year= 1993 | volume= 14 | issue= 4 | pages= 253-6 | pmid=8378657 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8378657  }} </ref><ref name="pmid25326572">{{cite journal| author=Arroyo-Ávila M, Vilá LM| title=Limited systemic sclerosis initially presenting with mesenteric panniculitis. | journal=BMJ Case Rep | year= 2014 | volume= 2014 | issue=  | pages=  | pmid=25326572 | doi=10.1136/bcr-2014-206961 | pmc=4202032 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25326572  }} </ref><ref name="pmid18405487">{{cite journal| author=Garrido A, Verdejo C, Márquez JL, Giráldez A, Trigo C, Belda O| title=[Intestinal lymphoma and mesenteric panniculitis: complications of undiagnosed celiac disease]. | journal=Gastroenterol Hepatol | year= 2008 | volume= 31 | issue= 4 | pages= 221-4 | pmid=18405487 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18405487  }} </ref>
<ref name="pmid14615565">{{cite journal| author=Horton KM, Lawler LP, Fishman EK| title=CT findings in sclerosing mesenteritis (panniculitis): spectrum of disease. | journal=Radiographics | year= 2003 | volume= 23 | issue= 6 | pages= 1561-7 | pmid=14615565 | doi=10.1148/rg.1103035010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14615565  }} </ref><ref name="pmid10705180">{{cite journal| author=Papadaki HA, Kouroumalis EA, Stefanaki K, Roussomoustakaki M, Daskalogiannaki ME, Reppa D et al.| title=Retractile mesenteritis presenting as fever of unknown origin and autoimmune haemolytic anaemia. | journal=Digestion | year= 2000 | volume= 61 | issue= 2 | pages= 145-8 | pmid=10705180 | doi=10.1159/000007748 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10705180  }} </ref><ref name="pmid11818670">{{cite journal| author=Vidarsson B, Matthíasson P, Agnarsson BA, Onundarson PT| title=Mesenteric panniculitis presenting with autoimmune haemolytic anaemia. | journal=Acta Haematol | year= 2002 | volume= 107 | issue= 1 | pages= 35-7 | pmid=11818670 | doi=10.1159/000046627 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11818670  }} </ref><ref name="pmid8832610">{{cite journal| author=Vernace MA, Bellucci AG, Mossey RT, Susin M, Mailloux LU, Wilkes BM et al.| title=Minimal change nephropathy associated with sclerosing mesenteritis. | journal=Nephron | year= 1996 | volume= 73 | issue= 3 | pages= 473-6 | pmid=8832610 | doi=10.1159/000189113 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8832610  }} </ref><ref name="pmid7103574">{{cite journal| author=Dor AM, Kohler JL, Aubrespy P, Scheiner C, Pizzi M, Lebreuil G| title=[Mesenteric panniculitis, an unusual initial stage of acute lupus erythematosus in a ten-year old girl (author's transl)]. | journal=Arch Anat Cytol Pathol | year= 1982 | volume= 30 | issue= 2 | pages= 121-4 | pmid=7103574 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7103574  }} </ref><ref name="pmid8378657">{{cite journal| author=Sauvaget F, Piette JC, Galezowski N, Jouanique C, Chapelon C, Blétry O et al.| title=[Relapsing polychondritis and mesenteric panniculitis: apropos of 2 cases]. | journal=Rev Med Interne | year= 1993 | volume= 14 | issue= 4 | pages= 253-6 | pmid=8378657 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8378657  }} </ref><ref name="pmid25326572">{{cite journal| author=Arroyo-Ávila M, Vilá LM| title=Limited systemic sclerosis initially presenting with mesenteric panniculitis. | journal=BMJ Case Rep | year= 2014 | volume= 2014 | issue=  | pages=  | pmid=25326572 | doi=10.1136/bcr-2014-206961 | pmc=4202032 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25326572  }} </ref><ref name="pmid18405487">{{cite journal| author=Garrido A, Verdejo C, Márquez JL, Giráldez A, Trigo C, Belda O| title=[Intestinal lymphoma and mesenteric panniculitis: complications of undiagnosed celiac disease]. | journal=Gastroenterol Hepatol | year= 2008 | volume= 31 | issue= 4 | pages= 221-4 | pmid=18405487 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18405487  }} </ref>
|Unknown exact etiology:
|Unknown exact [[etiology]]:
* Encompasses a spectrum of idiopathic primary inflammatory and fibrotic processes affecting mesentery
* Encompasses a [[spectrum]] of [[idiopathic]] primary inflammatory and fibrotic processes affecting [[mesentery]]
|Varying components of:
|Varying components of:
* Inflammation, fibrosis, and fat necrosis
* [[Inflammation]], [[fibrosis]], and [[fat necrosis]]
* Adipocyte necrosis (mesenteric lipodystrophy)  
* [[Adipocyte]] [[necrosis]] ([[mesenteric]] [[lipodystrophy]])  
* Chronic inflammatory state (mesenteric panniculitis)
* [[Chronic (medicine)|Chronic]] [[inflammatory]] state ([[mesenteric]] [[panniculitis]])
* Fibrosis (sclerosing mesenteritis)
* [[Fibrosis]] (sclerosing mesenteritis)
|_
|_
|
|
* Non-neoplastic inflammatory and fibrotic disease
* Non-[[neoplastic]] [[inflammatory]] and fibrotic [[disease]]
|
|
*'''Abdominal surgery/trauma:'''
*'''Abdominal surgery/trauma:'''
**Cholecystectomy
**[[Cholecystectomy]]
**Appendectomy
**[[Appendectomy]]
**Hysterectomy
**[[Hysterectomy]]
**Colectomy
**[[Colectomy]]
**Pneumatic jackhammer
**Pneumatic jackhammer
**Occult ileal perforation
**[[Occult]] [[Ileum|ileal]] [[perforation]]
* '''Autoimmunity:'''
* '''Autoimmunity:'''
**Riedel thyroiditis  
**[[Riedel's thyroiditis|Riedel thyroiditis]]
**Primary sclerosing cholangitis
**[[Primary sclerosing cholangitis]]
**Retroperitoneal fibrosis
**[[Retroperitoneal fibrosis]]
**Orbital pseudotumor  
**[[Orbit (anatomy)|Orbital]] pseudotumor  
**Autoimmune hemolytic anemia
**[[Autoimmune hemolytic anemia]]
**Minimal change nephropathy  
**Minimal change [[nephropathy]]
**Systemic lupus erythematosus
**[[Systemic lupus erythematosus]]
**Relapsing polychondritis
**[[Relapsing polychondritis]]
**Multifocal fibrosclerosis  
**[[Multifocal fibrosclerosis]]
**Limited systemic sclerosis
**Limited [[systemic sclerosis]]
**Celiac-associated T-cell lymphoma
**[[Celiac disease|Celiac]]-associated [[T-cell lymphoma]]
*'''Paraneoplastic''' '''syndrome:'''
*'''Paraneoplastic''' '''syndrome:'''
**Non-Hodgkin lymphoma(most common)
**[[Non-Hodgkin lymphoma]](most common)
**Breast cancer
**[[Breast cancer]]
**Carcinoid  
**[[Carcinoid syndrome|Carcinoid]]
**Melanoma  
**[[Melanoma]]
**Squamous carcinoma and adenocarcinoma of the lung  
**[[Squamous carcinoma]] and [[adenocarcinoma of the lung]]
**Renal carcinoma  
**[[Renal carcinoma]]
**Multiple myeloma
**[[Multiple myeloma]]
**Hepatocellular carcinoma
**[[Hepatocellular carcinoma]]
**Prostate adenocarcinoma  
**[[Prostate adenocarcinoma]]
**Ovarian carcinoma
**[[Ovarian carcinoma]]
**Endometrial carcinoma  
**[[Endometrial carcinoma]]
**Cervical carcinoma  
**[[Cervical carcinoma]]
**Angiosarcoma
**[[Angiosarcoma]]
**Gastrointestinal adenocarcinomas
**[[Gastrointestinal tract|Gastrointestinal]] [[adenocarcinomas]]
* '''Ischemia''' '''and''' '''infection history:'''
* '''Ischemia''' '''and''' '''infection history:'''
**Typhoid fever  
**[[Typhoid fever]]
**Dysentery  
**[[Dysentery]]
**Tuberculosis
**[[Tuberculosis]]
**Syphilis
**[[Syphilis]]
**Malaria  
**[[Malaria]]
**Influenza
**[[Influenza]]
**Rheumatic fever
**[[Rheumatic fever]]
|
|
* Mesentery
* [[Mesentery]]
|
|
* Asymptomatic
* [[Asymptomatic]]
* Abdominal pain
* [[Abdominal pain]]
* Nausea and vomiting
* [[Nausea and vomiting]]
* Altered bowel habits (Constipation or diarrhea)
* Altered [[bowel]] habits ([[constipation]] or [[diarrhea]])
* Weight loss  
* [[Weight loss]]
* Fever
* [[Fever]]
* Malaise  
* [[Malaise]]
* Abdominal tenderness  
* [[Abdominal tenderness]]
* Abdominal distension
* [[Abdominal distension]]
* Peritoneal inflammation and ascites (usually chylous)  
* [[Peritoneal inflammation]] and [[ascites]] (usually [[Chylous ascites|chylous]])  
* Pleural effusion
* [[Pleural effusion]]
* Protein-losing enteropathy (Fever, edema)
* [[Protein-losing enteropathy]] ([[fever]], [[edema]])
* Biliary obstruction (hemolytic anemia, jaundice)  
* [[Biliary obstruction]] ([[hemolytic anemia]], [[jaundice]])  
* Bowel obstruction  
* [[Bowel obstruction]]
* Obstructive uropathy/renal failure
* [[Obstructive uropathy]]/[[renal failure]]
* Chylous ascites  
* [[Chylous ascites]]
* Chronic mesenteric ischemia
* [[Chronic (medical)|Chronic]] [[mesenteric ischemia]]
* Anemia
* [[Anemia]]
* Hypoalbuminemia
* [[Hypoalbuminemia]]
* Mesenteric vascular occlusion (both arterial and venous)
* [[Mesenteric vascular occlusion]] (both [[arterial]] and [[venous]])
|Nomenclature used in the medical literature for idiopathic primary inflammatory and fibrotic processes of the mesentery is as follows:
|[[Nomenclature Codes|Nomenclature]] used in the [[medical literature]] for [[idiopathic]] primary [[inflammatory]] and fibrotic processes of the [[mesentery]] is as follows:
* Mesenteric lipodystrophy (predominant fat necrosis predominance)
* [[Mesenteric]] [[lipodystrophy]] (predominant [[fat necrosis]] predominance)
* Mesenteric fibrosis
* [[Mesenteric]] [[fibrosis]]
* Mesenteric panniculitis
* [[Mesenteric]] [[panniculitis]]
* Mesenteric lipodystrophy
* [[Mesenteric]] [[lipodystrophy]]
* Retractile mesenteritis
* Retractile mesenteritis
* Mesenteric Weber-Christian disease
* [[Mesenteric]] [[Weber-Christian disease]]
* Xanthogranulomatous mesenteritis
* [[Xanthogranulomatous inflammation|Xanthogranulomatous]] mesenteritis
* Mesenteric lipogranuloma
* [[Mesenteric]] [[Lipogranulomatosis|lipogranuloma]]
* Liposclerotic mesenteritis
* Liposclerotic mesenteritis
* Inflammatory pseudotumor
* [[Inflammatory]] pseudotumor
* Isolated lipodystrophy
* Isolated [[lipodystrophy]]
* Retroperitoneal xanthogranuloma
* [[Retroperitoneal]] [[xanthogranuloma]]
|-
|-
|[[Retroperitoneal fibrosis|'''Retroperitoneal fibrosis''']]<ref name="VaglioMaritati2016">{{cite journal|last1=Vaglio|first1=A.|last2=Maritati|first2=F.|title=Idiopathic Retroperitoneal Fibrosis|journal=Journal of the American Society of Nephrology|volume=27|issue=7|year=2016|pages=1880–1889|issn=1046-6673|doi=10.1681/ASN.2015101110}}</ref><ref name="pmid16427494">{{cite journal| author=Vaglio A, Salvarani C, Buzio C| title=Retroperitoneal fibrosis. | journal=Lancet | year= 2006 | volume= 367 | issue= 9506 | pages= 241-51 | pmid=16427494 | doi=10.1016/S0140-6736(06)68035-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16427494  }} </ref><ref name="pmid18858051">{{cite journal| author=ORMOND JK| title=Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. | journal=J Urol | year= 1948 | volume= 59 | issue= 6 | pages= 1072-9 | pmid=18858051 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18858051  }} </ref><ref name="pmid12365466">{{cite journal| author=van Bommel EF| title=Retroperitoneal fibrosis. | journal=Neth J Med | year= 2002 | volume= 60 | issue= 6 | pages= 231-42 | pmid=12365466 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12365466  }} </ref><ref name="pmid19593224">{{cite journal| author=Scheel PJ, Feeley N| title=Retroperitoneal fibrosis: the clinical, laboratory, and radiographic presentation. | journal=Medicine (Baltimore) | year= 2009 | volume= 88 | issue= 4 | pages= 202-7 | pmid=19593224 | doi=10.1097/MD.0b013e3181afc439 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19593224  }} </ref><ref name="pmid21733570">{{cite journal| author=Vaglio A, Palmisano A, Alberici F, Maggiore U, Ferretti S, Cobelli R et al.| title=Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial. | journal=Lancet | year= 2011 | volume= 378 | issue= 9788 | pages= 338-46 | pmid=21733570 | doi=10.1016/S0140-6736(11)60934-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21733570  }} </ref><ref name="pmid26106209">{{cite journal| author=Palmisano A, Urban ML, Corradi D, Cobelli R, Alberici F, Maritati F et al.| title=Chronic periaortitis with thoracic aorta and epiaortic artery involvement: a systemic large vessel vasculitis? | journal=Rheumatology (Oxford) | year= 2015 | volume= 54 | issue= 11 | pages= 2004-9 | pmid=26106209 | doi=10.1093/rheumatology/kev225 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26106209  }} </ref><ref name="pmid15121404">{{cite journal| author=Uibu T, Oksa P, Auvinen A, Honkanen E, Metsärinne K, Saha H et al.| title=Asbestos exposure as a risk factor for retroperitoneal fibrosis. | journal=Lancet | year= 2004 | volume= 363 | issue= 9419 | pages= 1422-6 | pmid=15121404 | doi=10.1016/S0140-6736(04)16100-X | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15121404  }} </ref><ref name="pmid841463">{{cite journal| author=Koep L, Zuidema GD| title=The clinical significance of retroperitoneal fibrosis. | journal=Surgery | year= 1977 | volume= 81 | issue= 3 | pages= 250-7 | pmid=841463 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=841463  }} </ref><ref name="pmid5903120">{{cite journal| author=Graham JR, Suby HI, LeCompte PR, Sadowsky NL| title=Fibrotic disorders associated with methysergide therapy for headache. | journal=N Engl J Med | year= 1966 | volume= 274 | issue= 7 | pages= 359-68 | pmid=5903120 | doi=10.1056/NEJM196602172740701 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5903120  }} </ref><ref name="pmid14257741">{{cite journal| author=UTZ DC, ROOKE ED, SPITTELL JA, BARTHOLOMEW LG| title=RETROPERITONEAL FIBROSIS IN PATIENTS TAKING METHYSERGIDE. | journal=JAMA | year= 1965 | volume= 191 | issue=  | pages= 983-5 | pmid=14257741 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14257741  }} </ref><ref name="pmid22498446">{{cite journal| author=Goenka AH, Shah SN, Remer EM| title=Imaging of the retroperitoneum. | journal=Radiol Clin North Am | year= 2012 | volume= 50 | issue= 2 | pages= 333-55, vii | pmid=22498446 | doi=10.1016/j.rcl.2012.02.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22498446  }} </ref><ref name="pmid25089862">{{cite journal| author=Goldoni M, Bonini S, Urban ML, Palmisano A, De Palma G, Galletti E et al.| title=Asbestos and smoking as risk factors for idiopathic retroperitoneal fibrosis: a case-control study. | journal=Ann Intern Med | year= 2014 | volume= 161 | issue= 3 | pages= 181-8 | pmid=25089862 | doi=10.7326/M13-2648 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25089862  }} </ref>
| style="background:#DCDCDC;" align="center" + |[[Retroperitoneal fibrosis|'''Retroperitoneal fibrosis''']]<ref name="VaglioMaritati2016">{{cite journal|last1=Vaglio|first1=A.|last2=Maritati|first2=F.|title=Idiopathic Retroperitoneal Fibrosis|journal=Journal of the American Society of Nephrology|volume=27|issue=7|year=2016|pages=1880–1889|issn=1046-6673|doi=10.1681/ASN.2015101110}}</ref><ref name="pmid16427494">{{cite journal| author=Vaglio A, Salvarani C, Buzio C| title=Retroperitoneal fibrosis. | journal=Lancet | year= 2006 | volume= 367 | issue= 9506 | pages= 241-51 | pmid=16427494 | doi=10.1016/S0140-6736(06)68035-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16427494  }} </ref><ref name="pmid18858051">{{cite journal| author=ORMOND JK| title=Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. | journal=J Urol | year= 1948 | volume= 59 | issue= 6 | pages= 1072-9 | pmid=18858051 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18858051  }} </ref><ref name="pmid12365466">{{cite journal| author=van Bommel EF| title=Retroperitoneal fibrosis. | journal=Neth J Med | year= 2002 | volume= 60 | issue= 6 | pages= 231-42 | pmid=12365466 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12365466  }} </ref><ref name="pmid19593224">{{cite journal| author=Scheel PJ, Feeley N| title=Retroperitoneal fibrosis: the clinical, laboratory, and radiographic presentation. | journal=Medicine (Baltimore) | year= 2009 | volume= 88 | issue= 4 | pages= 202-7 | pmid=19593224 | doi=10.1097/MD.0b013e3181afc439 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19593224  }} </ref><ref name="pmid21733570">{{cite journal| author=Vaglio A, Palmisano A, Alberici F, Maggiore U, Ferretti S, Cobelli R et al.| title=Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial. | journal=Lancet | year= 2011 | volume= 378 | issue= 9788 | pages= 338-46 | pmid=21733570 | doi=10.1016/S0140-6736(11)60934-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21733570  }} </ref><ref name="pmid26106209">{{cite journal| author=Palmisano A, Urban ML, Corradi D, Cobelli R, Alberici F, Maritati F et al.| title=Chronic periaortitis with thoracic aorta and epiaortic artery involvement: a systemic large vessel vasculitis? | journal=Rheumatology (Oxford) | year= 2015 | volume= 54 | issue= 11 | pages= 2004-9 | pmid=26106209 | doi=10.1093/rheumatology/kev225 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26106209  }} </ref><ref name="pmid15121404">{{cite journal| author=Uibu T, Oksa P, Auvinen A, Honkanen E, Metsärinne K, Saha H et al.| title=Asbestos exposure as a risk factor for retroperitoneal fibrosis. | journal=Lancet | year= 2004 | volume= 363 | issue= 9419 | pages= 1422-6 | pmid=15121404 | doi=10.1016/S0140-6736(04)16100-X | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15121404  }} </ref><ref name="pmid841463">{{cite journal| author=Koep L, Zuidema GD| title=The clinical significance of retroperitoneal fibrosis. | journal=Surgery | year= 1977 | volume= 81 | issue= 3 | pages= 250-7 | pmid=841463 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=841463  }} </ref><ref name="pmid5903120">{{cite journal| author=Graham JR, Suby HI, LeCompte PR, Sadowsky NL| title=Fibrotic disorders associated with methysergide therapy for headache. | journal=N Engl J Med | year= 1966 | volume= 274 | issue= 7 | pages= 359-68 | pmid=5903120 | doi=10.1056/NEJM196602172740701 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5903120  }} </ref><ref name="pmid14257741">{{cite journal| author=UTZ DC, ROOKE ED, SPITTELL JA, BARTHOLOMEW LG| title=RETROPERITONEAL FIBROSIS IN PATIENTS TAKING METHYSERGIDE. | journal=JAMA | year= 1965 | volume= 191 | issue=  | pages= 983-5 | pmid=14257741 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14257741  }} </ref><ref name="pmid22498446">{{cite journal| author=Goenka AH, Shah SN, Remer EM| title=Imaging of the retroperitoneum. | journal=Radiol Clin North Am | year= 2012 | volume= 50 | issue= 2 | pages= 333-55, vii | pmid=22498446 | doi=10.1016/j.rcl.2012.02.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22498446  }} </ref><ref name="pmid25089862">{{cite journal| author=Goldoni M, Bonini S, Urban ML, Palmisano A, De Palma G, Galletti E et al.| title=Asbestos and smoking as risk factors for idiopathic retroperitoneal fibrosis: a case-control study. | journal=Ann Intern Med | year= 2014 | volume= 161 | issue= 3 | pages= 181-8 | pmid=25089862 | doi=10.7326/M13-2648 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25089862  }} </ref>


|
|
[[Idiopathic]] (<nowiki/>70%) [<nowiki/>[[Ormond's disease]]] is an immune-mediated disease caused by:
[[Idiopathic]] (<nowiki/>70%) [<nowiki/>[[Ormond's disease]]] is an [[immune]]-mediated disease caused by:
* Other autoim<nowiki/>mune diseases
* Other [[Autoimmune diseases|autoim]]<nowiki/>[[Autoimmune diseases|mune diseases]]
* Immunoglobul<nowiki/>in G4-related disease (IgG4-RD)
* [[Immunoglobulin|Immunoglobul]]<nowiki/>[[Immunoglobulin|in]] G4-related [[disease]] (IgG4-RD)
* Chronic peri<nowiki/>aortitis
* [[Chronic (medical)|Chronic]] peri<nowiki/>aortitis
* Allele HLA‑D<nowiki/>RB1*03, D32 polymorphism of the gene encoding CCR5 (chemokine receptor)
* [[Allele]] HLA‑D<nowiki/>RB1*03, D32 [[polymorphism]] of the [[gene]] encoding [[CCR5]] ([[chemokine receptor]])
* TTCCAT haplo<nowiki/>type of the gene encoding CCL11/eotaxin‑1 (drives the recruitment of eosinophils)  
* TTCCAT haplo<nowiki/>type of the [[gene]] encoding [[CCL11]]/eotaxin‑1 (drives the [[Recruitment status|recruitment]] of [[eosinophils]])  
[[Secondary]] to:
[[Secondary]] to:
* Certain [[drugs]]
* Certain [[drugs]]
*Underlying [[malignancy]] such as [[lymphoma]]
*Underlying [[malignancy]] such as [[lymphoma]]
|Fibro-inflammatory disease histologically hallmarked by fibrous tissue and chronic inflammation:
|Fibro-[[inflammatory]] disease [[histologically]] hallmarked by [[fibrous tissue]] and [[Chronic (medical)|chronic]] [[inflammation]]:
* Fibrous tissue composed of extracellular matrix containing type I collagen fibers organized in thick irregular bundles, often encircling small retroperitoneal vessels  
* [[Fibrous tissue]] composed of [[extracellular matrix]] containing [[type I collagen]] fibers organized in thick irregular bundles, often encircling small [[retroperitoneal]] [[vessels]]
* Fibroblasts:
* [[Fibroblasts]]:
**Show signs of activation and transition into myofibroblasts (''α''‑smooth muscle actin expression)
**Show [[signs]] of activation and [[transition]] into [[myofibroblasts]] (''α''‑[[smooth muscle]] [[actin]] [[expression]])
**Are major source of collagen production
**Are major source of [[collagen]] production
**Rarely show mitoses, undergo clonal proliferation
**Rarely show [[mitoses]], undergo [[Clonal selection|clonal]] [[proliferation]]
*Inflammatory infiltrate consists of numerous lymphocytes, plasma cells, and macrophages
*[[Inflammatory]] infiltrate consists of numerous [[lymphocytes]], [[plasma cells]], and [[macrophages]]
*Inflammatory cells maybe:
*[[Inflammatory cells]] maybe:
**Interspersed within the collagen bundles (“diffuse” pattern) or
**Interspersed within the [[collagen]] bundles (“[[diffuse]]” [[pattern]]) or
**Organized in nodular aggregates, usually around small vessels, having a B cell core surrounded by CD4+ T cells
**Organized in [[nodular]] aggregates, usually around small [[vessels]], having a [[B cell]] core surrounded by [[CD4+]] [[T cells]]
*When IgG4+/total IgG+ plasma cell ratio is >40%, RPF is classified as “IgG4-related” if other features such as storiform fibrosis, eosinophil infiltration, and obliterative phlebitis are also present
*When IgG4+/total IgG+ [[plasma cell]] ratio is >40%, RPF is classified as “IgG4-related” if other features such as storiform [[fibrosis]], [[eosinophil]] [[Infiltration (medical)|infiltration]], and obliterative [[phlebitis]] are also present
|
|
* Tryptase positive (mast cells)
* [[Tryptase]] positive ([[mast cells]])
|
|
* Benign immune mediated inflammatory process
* [[Benign]] [[immune]] mediated [[inflammatory]] [[Process (anatomy)|process]]
|
|
*[[Idiopathic]] [<nowiki/>[[Ormond's disease]]]  
*[[Idiopathic]] [<nowiki/>[[Ormond's disease]]]  
*[[Secondary]] to certain [[drugs]] (ergot-derivatives, methysergide,  bromocriptine, beta blockers, methyldopa, hydralazine, analgesics)
*[[Secondary]] to certain [[drugs]] ([[ergot]]-derivatives, [[methysergide]][[bromocriptine]], [[beta blockers]], [[methyldopa]], [[hydralazine]], [[analgesics]])
*Biological agents (etanercept, infliximab)
*[[Biological agents]] ([[etanercept]], [[infliximab]])
*Underlying [[malignancy]] (carcinoid, Hodgkin and non-Hodgkin lymphoma, sarcomas, colorectal, breast, prostate and bladder carcinoma[[lymphoma|)]]
*Underlying [[malignancy]] ([[Carcinoid syndrome|carcinoid]], [[Hodgkin's lymphoma|Hodgkin]] and [[non-Hodgkin lymphoma]], [[sarcomas]], [[colorectal]], [[breast]], [[prostate]] and [[bladder carcinoma]][[lymphoma|)]]
*Infections (tuberculosis, histoplasmosis, actinomycosis)
*[[Infections]] ([[tuberculosis]], [[histoplasmosis]], [[actinomycosis]])
*Radiation therapy for testicular seminoma, colon and pancreatic cancer
*[[Radiation therapy]] for [[testicular seminoma]], [[Colon (anatomy)|colon]] and [[pancreatic cancer]]
*Retroperitoneal hemorrhage
*[[Retroperitoneal hemorrhage]]
*Surgery (lymphadenectomy, colectomy, aortic aneurysmectomy)
*[[Surgery]] ([[lymphadenectomy]], [[colectomy]], [[aortic]] [[aneurysm]]<nowiki/>ectomy)
*Secondary (AA) amyloidosis
*[[Secondary]] (AA) [[amyloidosis]]
*Trauma
*[[Trauma]]
*Barium enema
*[[Barium enema]]
*Mesenteric panniculitis
*[[Mesenteric]] [[panniculitis]]
*Different forms of histiocytosis particularly Erdheim-Chester disease  
*Different forms of [[histiocytosis]] particularly [[Erdheim-Chester disease]]
*Exposure to asbestos
*Exposure to [[asbestos]]
*Tobacco smoke exposure
*[[Tobacco smoke]] exposure
|
|
* Thoracic aorta  
* [[Thoracic aorta]]
* Mesenteric arteries
* [[Mesenteric]] [[arteries]]
* Renal arteries and veins
* [[Renal arteries]] and [[veins]]
* Epiaortic arteries
* Epiaortic [[arteries]]
* Ureters
* [[Ureters]]
* Abdominal organs
* [[Abdominal organs]]
* Pancreas
* [[Pancreas]]
* Salivary glands
* [[Salivary glands]]
* Lymph nodes
* [[Lymph nodes]]
* Pituitary gland  
* [[Pituitary gland]]
|
|
* Pain in the lower back, abdomen, or flank (most common presentation,  bilateral or unilateral flank pain that radiates to the inguinal region, or acute onset pain similar to renal colic)
* [[Pain]] in the [[lower back]], [[abdomen]], or [[flank]] (most common presentation,  bilateral or unilateral [[flank]] [[pain]] that radiates to the [[inguinal region]], or [[acute]] onset [[pain]] similar to [[renal colic]])
* Abdominal aortic aneurysms
* [[Abdominal aortic aneurysm|Abdominal aortic aneurysms]]
* Malaise
* [[Malaise]]
* Anorexia
* [[Anorexia]]
* Weight loss
* [[Weight loss]]
* Fever
* [[Fever]]
* Nausea and vomiting
* [[Nausea and vomiting]]
* Testicular pain
* [[Testicular pain]]
* Constipation
* [[Constipation]]
* Venous compression (inferior vena cava) leading to:
* [[Venous compression ultrasound|Venous compression]] ([[inferior vena cava]]) leading to:
**Lower limb edema (lymphatic compression)
**[[Lower limb]] [[edema]] ([[Lymphatic system|lymphatic]] compression)
**Inferior vena cava syndrome
**[[Inferior vena cava syndrome]]
**Deep vein thrombosis
**[[Deep vein thrombosis]]
**Pulmonary embolism  
**[[Pulmonary embolism]]
* Arterial stenosis (rare) causing:
* [[Arterial]] [[stenosis]] (rare) causing:
**Upper-leg claudication
**Upper-[[leg]] [[claudication]]
**Mesenteric ischemia (mesenteric arteries compression)  
**[[Mesenteric ischemia]] ([[mesenteric arteries]] compression)  
**Thoracic aorta and/or the epi-aortic arteries' compression
**[[Thoracic aorta]] and/or the epi-aortic [[arteries]]' compression
*Recurrent laryngeal nerve paralysis causing hoarsness and  dry cough
*[[Recurrent laryngeal nerve]] [[paralysis]] causing [[hoarseness]] and  [[dry cough]]
*Acute kidney injury (AKI) (due to bilateral encasement of ureteral and renal involvement in peri-iliac retroperitoneal fibrosis)
*[[Acute kidney injury]] (AKI) (due to bilateral encasement of [[ureteral]] and [[renal]] involvement in peri-iliac [[retroperitoneal fibrosis]])
*Renal atrophy (due to previous transient unilateral obstruction or renal artery stenosis)
*[[Renal]] [[atrophy]] (due to previous [[transient]] unilateral [[obstruction]] or [[renal artery stenosis]])
*Obstructive uropathy
*[[Obstructive uropathy]]
*Gross hematuria
*Gross [[hematuria]]
*Urinary symptomes (urinary urgency,frequency, and dysuria)  
*[[Urinary symptoms]] ([[urinary urgency]],[[frequency]], and [[dysuria]])  
*Hypertension (due to renal artery impingement)  
*[[Hypertension]] (due to [[renal artery]] [[Impingement syndrome|impingement]])  
*Obstruction of the inferior vena cava and/or iliac veins
*[[Obstruction]] of the [[inferior vena cava]] and/or [[iliac]] [[veins]]
*Thrombophlebitis
*[[Thrombophlebitis]]
*Deep vein thrombosis  
*[[Deep vein thrombosis]]
*Varicocele
*[[Varicocele]]
*Hydrocele
*[[Hydrocele]]
|Retroperitoneal fibrosis is also known as:
|[[Retroperitoneal fibrosis]] is also known as:
* Ormond's disease  
* [[Ormond's disease]]
* Periureteritis fibrosa
* Periureteritis fibrosa
* Periureteritis plastica
* Periureteritis plastica
* Chronic periureteritis
* [[Chronic (medical)|Chronic]] periureteritis
* Sclerosing retroperitoneal granuloma
* Sclerosing [[retroperitoneal]] [[granuloma]]
* Fibrous retroperitonitis
* [[Fibrous]] retroperitonitis
|-
|-
|[[Lymphoma|'''Lymphoma''']]
| style="background:#DCDCDC;" align="center" + |[[Lymphoma|'''Lymphoma''']]
|
|
* '''Diffuse large B cell lymphoma (DLBCL)''':
* '''[[Diffuse Large B-Cell Lymphoma|Diffuse large B cell lymphoma (DLBCL)]]''':
** Rare ''MALT1'' rearrangements, trisomy 18 in some, possible NFκB activation in minority  
** [[Rare]] ''[[MALT1]]'' [[Rearrangement|rearrangements]], [[trisomy 18]] in some, possible [[NFκB]] [[Activation energy|activation]] in [[minority]]
* '''Extranodal marginal B cell lymphoma''' arising from mucosal associated lymphoid tissue (MALT lymphoma):
* '''Extranodal marginal [[B cell lymphoma]]''' arising from [[Mucosal associated lymphoid tissue lymphoma|mucosal associated lymphoid tissue]] ([[MALT lymphoma]]):
** Clonal rearrangements of immunoglobulin genes
** [[Clonal selection|Clonal]] [[Rearrangement|rearrangements]] of [[immunoglobulin]] [[genes]]
** Often trisomy of 3, 12, 18
** Often [[trisomy]] 3, 12, [[Trisomy 18|18]]
** Usually negative for characteristic ''MALT1'' rearrangements t(11;18)(q21;q21) and t(14;18)(q32;q21)  
** Usually negative for [[Characteristic impedance|characteristic]] ''[[MALT1]]'' [[Rearrangement|rearrangements]] t(11;18)(q21;q21) and t(14;18)(q32;q21)  
* '''Follicular lymphoma:'''
* '''[[Follicular lymphoma]]:'''
** Presence of t(14;18)(q32;q21) translocation has not been systematically assessed in primary breast follicular lymphoma
** Presence of t(14;18)(q32;q21) [[translocation]] has not been systematically assessed in primary [[breast]] [[follicular lymphoma]]
* '''Burkitt lymphoma:'''
* '''[[Burkitt lymphoma]]:'''
** t(8;14)(q24;q32) ''c-Myc'' and ''IgH'' translocation is most frequent
** t(8;14)(q24;q32) ''c-[[Myc]]'' and ''IgH'' [[Translocations|translocation]] is most frequent
** t(2;8)(p12;q24) ''IgΚ'' and ''c-Myc''
** t(2;8)(p12;q24) ''IgΚ'' and ''c-[[Myc]]''
** t(8;22)(q24;q11) ''c-Myc'' and ''Igλ''
** t(8;22)(q24;q11) ''c-[[Myc]]'' and ''Igλ''
|'''Diffuse large B cell lymphoma (DLBCL):'''
|'''[[Diffuse large B cell lymphoma]] ([[Diffuse large B cell lymphoma|DLBCL]]):'''
* Diffuse infiltration by large lymphoid cells, centroblastic > immunoblastic, activated B cell type > germinal center type
* [[Diffuse]] [[Infiltration (medical)|infiltration]] by large [[Lymphoid cell|lymphoid cells]], [[Centroblastic and centrocytic lymphoma|centroblastic]] > [[Immunoblastic Lymphadenopathy|immunoblastic]], activated [[B cell]] type > [[germinal center]] type
'''Extranodal marginal B cell lymphoma:'''
'''Extranodal marginal [[B cell lymphoma]]:'''
* Vaguely nodular growth pattern, neoplastic cells occupy the marginal zone surrounding the B cell follicle mantle zone but can have diffuse growth pattern if follicles infiltrated / replaced
* Vaguely [[nodular]] [[growth]] [[pattern]], [[neoplastic]] [[Cells (biology)|cells]] occupy the [[marginal zone]] surrounding the [[B cell]] [[follicle]] [[mantle zone]] but can have [[diffuse]] [[growth]] [[pattern]] if [[Follicle|follicles]] infiltrated / replaced
* Marginal zone B cells, variable plasma cells, reactive follicles (±)
* [[Marginal zone]] [[B cells]], [[variable]] [[plasma cells]], reactive [[Follicle|follicles]] (±)
* Monocytoid-like cells
* [[Monocytoid]]-like [[Cells (biology)|cells]]
* Plasmacytic differentiation
* Plasmacytic [[differentiation]]
* Low mitotic rate
* Low [[mitotic]] [[rate]]
'''Follicular lymphoma:'''
'''[[Follicular lymphoma]]:'''
* Follicular and diffuse architectural patterns
* [[Follicular lymphoma|Follicular]] and [[diffuse]] architectural [[Pattern|patterns]]
* Monotonous population of small cleaved cells (centrocytes)
* Monotonous [[population]] of small cleaved [[Cells (biology)|cells]] (centrocytes)
* Variable numbers of centroblasts (large noncleaved cells)
* Variable numbers of centroblasts (large noncleaved [[Cells (biology)|cells]])
* Associated sclerosis can cause single file infiltration pattern
* Associated [[sclerosis]] can cause single file [[Infiltration (medical)|infiltration]] [[pattern]]
'''Burkitt lymphoma:'''
'''[[Burkitt's lymphoma|Burkitt lymphoma]]:'''
* Sheets of uniform medium sized lymphocytes with round nuclei, multiple basophilic nucleoli, course chromatin, scant to moderate basophilic cytoplasm, minimal intervening stroma
* Sheets of [[Uniform distribution|uniform]] medium [[Size consistency|sized]] [[lymphocytes]] with round [[nuclei]], [[Multiple-conclusion logic|multiple]] [[basophilic]] [[nucleoli]], [[Chromatin|coarse chromatin]], scant to moderate [[basophilic]] [[cytoplasm]], minimal intervening [[stroma]]
* High mitotic rate
* High [[mitotic]] [[rate]]
* Tingible body macrophages containing apoptotic debris produce a "starry sky" appearance
* Tingible [[body]] [[macrophages]] containing [[apoptotic]] [[Debrisoquine|debris]] produce a "starry sky" [[appearance]]
* '''Mantle cell lymphoma:'''
* '''[[Mantle cell lymphoma]]:'''
* Small to medium sized, slightly irregular cells with scant cytoplasm
* Small to medium [[Size consistency|sized]], slightly irregular [[Cells (biology)|cells]] with scant [[cytoplasm]]
* '''Classic Hodgkin lymphoma:'''
* '''Classic [[Hodgkin lymphoma]]:'''
* Reed-Sternberg cells and variants in a reactive background
* [[Reed-Sternberg cells]] and variants in a reactive [[background]]
* '''Nodular lymphocyte predominant Hodgkin lymphoma:'''
* '''[[Nodular]] [[lymphocyte]] predominant [[Hodgkin's lymphoma|Hodgkin lymphoma]]:'''
* Vague nodules of small B cells and interspersed large tumor cells (LP cells) with thin nuclear membranes, fine chromatin and variable nucleoli
* [[Vagueness|Vague]] [[nodules]] of small [[B cells]] and interspersed large [[Tumor cell|tumor cells]] ([[LP]] [[Cells (biology)|cells]]) with thin [[Nuclear membrane|nuclear membranes]], fine [[chromatin]] and variable [[nucleoli]]
* '''MALT lymphoma''':
* '''[[MALT lymphoma]]''':
** Infiltration of thyroid epithelium creates lymphoepithelial lesions (lymphocytes "stuff" glandular lumina)
** [[Infiltration (medical)|Infiltration]] of [[thyroid]] [[epithelium]] creates [[Lymphoepithelioma|lymphoepithelial]] [[lesions]] ([[lymphocytes]] "stuff" [[glandular]] [[Luminal|lumina]])
** May have background lymphocytic thyroiditis
** May have [[background]] [[lymphocytic thyroiditis]]
|'''Positive for:'''
|'''Positive for:'''
* '''Diffuse large B cell lymphoma (DLBCL):'''
* '''[[Diffuse large B cell lymphoma]] ([[Diffuse large B cell lymphoma|DLBCL]]):'''
** CD45, CD20, CD79a, PAX5, BCL2, Ki67 (high)
** [[CD45]], [[CD20]], [[CD79a]], [[PAX5]], [[BCL2-like 1|BCL2]], [[Ki-67 (Biology)|Ki67]] (high)
** Activated B cell type (CD10-, BCL6- or CD10-, BCL6+, MUM1 / IRF+) is more common
** Activated [[B cell]] type ([[CD10]]-, [[BCL6]]- or [[CD10]]-, [[BCL6]]+, MUM1 / [[IRF1|IRF]]+) is more common
** Germinal center type (CD10+, BCL6+ or CD10-, BCL6+, MUM1 / IRF-)  
** [[Germinal center]] type ([[CD10]]+, [[BCL6]]+ or [[CD10]]-, [[BCL6]]+, MUM1 / [[IRF2|IRF]]-)  
** Surface Ig+
** [[Surface area|Surface]] [[IgA|Ig]]+
** CD43±
** [[CD43]]±
* '''Extranodal marginal B cell lymphoma''' arising from mucosal associated lymphoid tissue (MALT lymphoma):
* '''Extranodal marginal [[B cell lymphoma]]''' arising from [[Mucosal associated lymphoid tissue lymphoma|mucosal associated lymphoid tissue]] ([[MALT lymphoma]]):
** Surface and cytoplasmic Ig+ (IgM > IgG or IgA)
** [[Surface anatomy|Surface]] and [[cytoplasmic]] [[IgA|Ig]]+ ([[Immunoglobulin M|IgM]] > [[Immunoglobulin G|IgG]] or [[IgA]])
** CD45, CD20, CD79a, PAX5, variable CD43, variable BCL2, Ki67 (low)
** [[CD45]], [[CD20]], [[CD79a]], [[PAX5]], [[variable]] [[CD43]], [[variable]] [[BCL2-like 1|BCL2]], [[Ki67]] (low)
** Variable monotypic cytoplasmic '''immunoglobulin''' seen in cases with plasmacytic differentiation
** [[Variable]] monotypic [[cytoplasmic]] '''[[immunoglobulin]]''' seen in cases with plasmacytic [[differentiation]]
** CD21 and CD23 highlight expanded / disrupted follicular dendritic meshworks
** [[CD21]] and [[CD23]] highlight [[Expanded octet|expanded]] / disrupted follicular [[Dendritic cells|dendritic]] meshworks
** CD43±
** [[CD43]]±
* '''Follicular lymphoma:'''
* '''[[Follicular lymphoma]]:'''
** Surface Ig+
** [[Surface anatomy|Surface]] [[IgA|Ig]]+
** CD45, CD20, CD79a, CD10, BCL6, BCL2
** [[CD45]], [[CD20]], [[CD79a]], [[CD10]], [[BCL6]], [[BCL2-like 1|BCL2]]
** CD21 and CD23 highlight follicular dendritic cell meshworks
** [[CD21]] and [[CD23]] highlight follicular dendritic [[cell]] meshworks
* '''Burkitt lymphoma:'''
* '''[[Burkitt's lymphoma|Burkitt lymphoma]]:'''
** Surface IgM+
** [[Surface anatomy|Surface]] [[Immunoglobulin M|IgM]]+
** CD45, CD20, CD79a, PAX5, CD10, BCL6, Ki67 (~100%)
** [[CD45]], [[CD20]], [[CD79a]], [[PAX5]], [[CD10]], [[BCL6]], [[Ki67]] (~100%)
* '''Mantle cell lymphoma:'''
* '''[[Mantle cell lymphoma]]:'''
** Surface IgMD+, CD20+, CD5+, CD10-, CD43+, cyclin D1+
** [[Surface anatomy|Surface]] IgMD+, [[CD20]]+, [[CD5]]+, [[CD10]]-, [[CD43]]+, [[cyclin D1]]+
* '''Classic Hodgkin lymphoma:'''
* '''Classic [[Hodgkin's lymphoma|Hodgkin lymphoma]]:'''
** R-S cells: CD30+, CD15±, PAX5+
** [[Reed-Sternberg cell|R-S cells]]: [[CD30]]+, [[CD15]]±, [[PAX5]]+
* '''Nodular lymphocyte predominant Hodgkin lymphoma:'''
* '''[[Nodular]] [[lymphocyte]] predominant [[Hodgkin's lymphoma|Hodgkin lymphoma]]:'''
** LP cells: CD45+, CD20+
** [[LP|LP cells]]: [[CD45]]+, [[CD20]]+
* '''Thyroid MALT lymphoma:'''
* '''[[Thyroid]] [[MALT lymphoma]]:'''
** CD20
** [[CD20]]
** Keratin  
** [[Keratin]]
** Thyroglobulin
** [[Thyroglobulin]]
** CD45
** [[CD45]]
* '''Anaplastic large T cell lymphoma:'''  
* '''[[Anaplastic]] large [[T cell lymphoma]]:'''  
** CD3, CD10, CD30, CD43, CD99, TdT
** [[CD3]], [[CD10]], [[CD30]], [[CD43]], [[CD99]], TdT
* '''PreT lymphoblastic lymphoma:'''  
* '''[[Lymphoblastic lymphoma|PreT lymphoblastic lymphoma]]:'''  
** CD3, CD10, CD99, TdT
** [[CD3]], [[CD10]], [[CD99]], TdT
'''Negative for:'''
'''Negative for:'''
* '''Diffuse large B cell lymphoma (DLBCL):'''
* '''[[Diffuse large B cell lymphoma]] ([[DLBCL]]):'''
** EBER
** EBER
* '''Extranodal marginal B cell lymphoma, MALT type:'''
* '''Extranodal marginal [[B-cell lymphoma|B cell lymphoma]], [[MALT lymphoma|MALT]] type:'''
** CD5, CD10, CD23, BCL6, cyclin D1
** [[CD5]], [[CD10]], [[CD23]], [[BCL6]], [[cyclin D1]]
* '''Follicular lymphoma:'''
* '''[[Follicular lymphoma]]:'''
** CD5, CD23, cyclin D1
** [[CD5]], [[CD23]], [[cyclin D1]]
* '''Burkitt lymphoma:'''
* '''[[Burkitt's lymphoma|Burkitt lymphoma]]:'''
** CD5, CD23, TdT, BCL2
** [[CD5]], [[CD23]], TdT, [[BCL2-like 1|BCL2]]
* '''Mantle cell lymphoma:'''
* '''[[Mantle cell lymphoma]]:'''
** CD10-
** [[CD10]]-
* '''Classic Hodgkin lymphoma:'''
* '''Classic [[Hodgkin lymphoma]]:'''
** R-S cells: ALK-, CD45-, CD3-
** [[Reed-Sternberg cells|R-S cells]]: [[ALK-positive ALCL|ALK]]-, [[CD45]]-, [[CD3]]-
* '''Nodular lymphocyte predominant Hodgkin lymphoma:'''
* '''[[Nodular]] [[lymphocyte]] predominant [[Hodgkin lymphoma]]:'''
** LP cells: CD15-, CD30-
** [[LP]] [[Cells (biology)|cells]]: [[CD15]]-, [[CD30]]-
* '''Anaplastic large T cell lymphoma:'''  
* '''[[Anaplastic]] large [[T cell lymphoma]]:'''  
** ALK1, bcl6
** [[ALK-positive ALCL|ALK]]1, bcl6
* '''PreT lymphoblastic lymphoma:'''
* '''PreT [[lymphoblastic lymphoma]]:'''
** Bcl6-
** Bcl6-
|
|
* Malignant
* [[Malignant]]
|
|
* MALT lymphoma (Extranodal marginal B cell lymphoma may arise from it)
* [[MALT lymphoma]] (Extranodal marginal [[B-cell lymphoma|B cell lymphoma]] may arise from it)
* Underlying autoimmune disease
* Underlying [[autoimmune disease]]
* EBV (endemic and sporadic Burkitt lymphoma)
* [[Epstein Barr virus|EBV]] ([[endemic]] and sporadic [[Burkitt's lymphoma|Burkitt lymphoma]])
* HIV (immunodeficiency associated Burkitt lymphoma)
* [[Human Immunodeficiency Virus (HIV)|HIV]] ([[immunodeficiency]] associated [[Burkitt's lymphoma|Burkitt lymphoma]])
* HHV8
* [[HHV-8]]
* Chronic hepatitis C infection
* [[Chronic (medical)|Chronic]] [[hepatitis C infection]]
* Transplants
* [[Transplants In Mind|Transplants]]
* Chemotherapy
* [[Chemotherapy]]
*  
*  
|Anywhere in body
|Anywhere in [[Human body|body]]
* Lymph nodes
* [[Lymph nodes]]
* Esophagus
* [[Esophagus]]
* Bladder
* [[Urinary bladder|Bladder]]
* Bone
* [[Bone]]
* Oral cavity
* [[Oral cavity]]
* Thyroid gland
* [[Thyroid gland]]
|
|
* Painless palpable mass, with or without ipsilateral lymphadenopathy
* Painless [[palpable]] [[mass]], with or without [[ipsilateral]] [[lymphadenopathy]]
* Fever
* [[Fever]]
* Night sweats
* [[Night sweats]]
* Weight loss
* [[Weight loss]]
* Neck mass
* [[Neck masses|Neck mass]]
* Dysphagia  
* [[Dysphagia]]
* Odynophagia
* [[Odynophagia]]
* Chest / abdominal pain
* [[Chest]] / [[abdominal pain]]
* Weight loss
* [[Weight loss]]
* Hemorrhage
* [[Hemorrhage]]
* Vocal cord paralysis / hoarseness
* [[Vocal cord paralysis]] / [[hoarseness]]
* Stricture / obstruction
* [[Stricture]] / [[obstruction]]
* Perforation with esophagomediastinal or esophagotracheobronchial fistula or mediastinitis
* [[Perforation]] with esophagomediastinal or esophagotracheobronchial [[fistula]] or [[mediastinitis]]
* Vocal cord paralysis
* [[Cold]] [[Nodule (medicine)|nodule]]
* Cold nodule
*  
*  
|WHO classification of lymphoma:
|[[World Health Organization|WHO]] [[classification]] of [[lymphoma]]:
* '''Hodgkin lymphomas''' arise from precursor B cells (Reed-Sternberg cells)
* '''[[Hodgkin's lymphoma|Hodgkin lymphomas]]''' arise from [[precursor]] [[B cells]] ([[Reed-Sternberg cells]])
** '''Classic Hodgkin lymphoma (CHL)''' - 95% is further subclassified as:
** '''Classic [[Hodgkin lymphoma]] (CHL)''' - 95% is further subclassified as:
*** Nodular sclerosis
*** [[Nodular sclerosis]]
*** Lymphocyte rich
*** [[Lymphocyte]] [[Rich focus|rich]]
*** Mixed cellularity
*** [[Mixed-handed|Mixed]] cellularity
*** Lymphocyte depleted
*** [[Lymphocyte]] depleted
** '''Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL)''' - 5%
** '''[[Nodular]] [[lymphocyte]] predominant [[Hodgkin lymphoma]] (NLPHL)''' - 5%
* '''Non-Hodgkin lymphomas''' arise from monoclonal expansion of malignant B or T cells
* '''[[Non-Hodgkin lymphomas]]''' arise from monoclonal [[Expansion ratio|expansion]] of [[malignant]] [[B cell|B]] or [[T cells]]
** ''B cell lymphomas''
** ''[[B-cell lymphoma|B cell lymphomas]]''
*** B cell acute lymphoblastic lymphoma (ALL)
*** [[B cell]] [[Acute (medicine)|acute]] [[lymphoblastic lymphoma]] ([[ALL]])
*** Chronic lymphocytic lymphoma / small lymphocytic leukemia (CLL / SLL)
*** [[Chronic (medical)|Chronic]] [[lymphocytic lymphoma]] / small [[lymphocytic leukemia]] ([[Chronic lymphocytic leukemia|CLL]] / [[SLL]])
*** Mantle cell lymphoma
*** [[Mantle cell lymphoma]]
*** Follicular lymphoma
*** [[Follicular lymphoma]]
*** Marginal zone B cell lymphoma
*** [[Marginal zone lymphoma|Marginal zone B cell lymphoma]]
*** Extranodal MALT type
*** Extranodal [[MALT lymphoma|MALT]] type
*** Hairy cell leukemia
*** [[Hairy cell leukemia]]
*** Plasmacytoma / plasma cell myeloma
*** [[Plasmacytoma]] / [[Plasma cell neoplasm|plasma cell myeloma]]
*** Diffuse large B cell lymphoma (DLBCL)
*** [[Diffuse large B cell lymphoma]] ([[Diffuse large B cell lymphoma|DLBCL]])
*** Burkitt lymphoma
*** [[Burkitt's lymphoma|Burkitt lymphoma]]
** ''T / NK cell lymphomas''
** ''[[T cell|T]] / [[NK cell]] [[lymphomas]]''
*** T cell acute lymphoblastic lymphoma (ALL)
*** [[T cell]] [[Acute (medicine)|acute]] [[lymphoblastic lymphoma]] ([[ALL]])
*** T cell CLL
*** [[T cell]] [[CLL]]
*** Mycosis fungoides / Sézary syndrome
*** [[Mycosis fungoides]] / [[Sezary syndrome|Sézary syndrome]]
*** Peripheral T cell lymphoma
*** Peripheral [[T-cell lymphoma|T cell lymphoma]]
*** Angioimmunoblastic T cell lymphoma
*** [[Angioimmunoblastic T-cell lymphoma|Angioimmunoblastic T cell lymphoma]]
*** Enteropathy associated intestinal T cell lymphoma
*** [[Enteropathy-associated T-cell lymphoma|Enteropathy associated intestinal T cell lymphoma]]
*** Hepatosplenic T cell lymphoma
*** [[Hepatosplenic T cell lymphoma]]
*** Anaplastic large cell lymphoma (ALCL)
*** [[Anaplastic large cell lymphoma]] ([[Anaplastic large cell lymphoma|ALCL]])
*** Extranodal NK / T cell lymphoma, nasal type
*** Extranodal [[Natural killer cell|NK]] / [[T cell lymphoma]], [[nasal]] type
|-
|-
|[[Rhabdomyosarcoma|'''Rhabdomyosarcoma''']] (RMS)<ref>{{Cite journal
| style="background:#DCDCDC;" align="center" + |[[Rhabdomyosarcoma|'''Rhabdomyosarcoma''']] ([[RMS]])<ref>{{Cite journal
  | author = [[H. M. Maurer]], [[M. Beltangady]], [[E. A. Gehan]], [[W. Crist]], [[D. Hammond]], [[D. M. Hays]], [[R. Heyn]], [[W. Lawrence]], [[W. Newton]] & [[J. Ortega]]
  | author = [[H. M. Maurer]], [[M. Beltangady]], [[E. A. Gehan]], [[W. Crist]], [[D. Hammond]], [[D. M. Hays]], [[R. Heyn]], [[W. Lawrence]], [[W. Newton]] & [[J. Ortega]]
  | title = The Intergroup Rhabdomyosarcoma Study-I. A final report
  | title = The Intergroup Rhabdomyosarcoma Study-I. A final report
Line 977: Line 973:
      
      
|
|
* ''MyoD1'' and ''PIK3CA'' mutation (sclerosing and spindle cell RMS)
* ''MyoD1'' and ''PIK3CA'' [[mutation]] (sclerosing and [[Spindle cells|spindle cell]] [[RMS]])
*Most common translocation is t(2;13)(q35;q14), causing''PAX3'<nowiki/>'''FOXO1'''''<nowiki/>''' gene fusion (alveolar RMS)'''
*Most common [[Translocations|translocation]] is t(2;13)(q35;q14), causing ''[[PAX3]]<nowiki/>'<nowiki/>'''[[FOXO1|FOXO]]'''''<nowiki/>'''''[[FOXO1|1]]'' [[gene fusion]] ([[alveolar]] [[RMS]])'''
*t(1;13)(p36;q14), causing''PAX7'<nowiki/>'''FOXO1'''''<nowiki/>''' gene fusion'''
*t(1;13)(p36;q14), causing ''[[PAX7]]<nowiki/>'<nowiki/>'''[[FOXO1|FOXO]]'''''<nowiki/>'''''[[FOXO1|1]]'' [[gene fusion]]'''
*Embryonal RMS is associated with:
*Embryonal [[RMS]] is associated with:
**Loss of heterozygosity (LOH) at 11p15 locus (site of ''IGF-2'' gene)
**[[Loss of heterozygosity]] (LOH) at 11p15 [[locus]] (site of ''IGF-2'' [[gene]])
**Other genes at 11p15.5 locus:
**Other [[genes]] at 11p15.5 [[locus]]:
***''H19''
***''[[H19 (gene)|H19]]''
***''CDKN1C''
***''[[CDKN1C]]''
***''HOTS''
***''HOTS''
*Other mutations include ''pRb'', ''TP53'', ''GLI'', ''CDKN2A'', ''CDKN2B'', ''RAS'', ''FGFR4'', ''PIK3CA'', ''CTNNB1'' [beta-catenin] and ''NF1'' genes
*Other [[mutations]] include ''[[PRb protein|pRb]]'', ''[[TP53]]'', ''[[GLI1|GLI]]'', ''[[CDKN2A]]'', ''[[CDKN2B]]'', ''[[RAS]]'', ''FGFR4'', ''PIK3CA'', ''CTNNB1'' [<nowiki/>[[beta-catenin]]] and ''[[NF1]]'' [[genes]]
*''VGLL2''and''NCOA2''rearrangements
*''VGLL2'' and ''NCOA2'' rearrangements
*Gene fusions include ''VGLL2-CITED2'', ''VGLL2-NC0A2'', ''TEAD1-NCOA2'', ''PAX3-NCOA2'', ''PAX3-NCOA2'', and ''SRF-NCOA2''.
*[[Gene fusion|Gene fusions]] include ''VGLL2-CITED2'', ''VGLL2-NC0A2'', ''TEAD1-NCOA2'', ''[[PAX3]]-NCOA2'', ''[[PAX3]]-NCOA2'', and ''SRF-NCOA2''
*Further gene mutations include:
*Further [[Gene mutation|gene mutations]] include:
**''RAS''
**''[[RAS]]''
**''RB1''
**''[[RB1]]''
**''PTCH''
**''[[PTCH1|PTCH]]''
**''MDM2''
**''[[MDM2]]''
**''CDK4''
**''[[CDK4]]''
**''MYCN''
**''MYCN''
**Basic helix-loop-helix [bHLH]  
**[[Basic helix-loop-helix]] [<nowiki/>[[bHLH]]]  
|'''Histologic classification:'''
|'''[[Histologic]] [[classification]]:'''
* Embryonal RMS comprises of:
* Embryonal [[RMS]] comprises of:
**Rhabdomyoblasts arranged in sheets and large nests, with infrequent intermixed fusiform cells
**Rhabdomyoblasts arranged in sheets and large nests, with infrequent intermixed fusiform [[Cells (biology)|cells]]
**Myofilaments with cross-striations
**[[Myofilament|Myofilaments]] with cross-striations
* Botryoid (sarcoma botryoides) and spindle cell (leiomyomatous) RMS:
* [[Botryoid rhabdomyosarcoma|Botryoid]] ([[sarcoma botryoides]]) and [[Spindle cells|spindle cell]] (leiomyomatous) [[RMS]]:
**"Grape-like" gross appearance of the tumor
**"Grape-like" [[gross]] [[appearance]] of the [[tumor]]
**Polypoid mass grows beneath an epithelial surface, with dense subepithelial aggregates of rhabdomyoblasts (the so-called "cambrium" layer)
**Polypoid [[mass]] grows beneath an [[epithelial]] [[Surface anatomy|surface]], with [[dense]] subepithelial aggregates of rhabdomyoblasts (the so-called "cambrium" layer)
* Sclerosing and spindle cell RMS
* Sclerosing and [[Spindle cells|spindle cell]] [[RMS]]
* Alveolar RMS comprises of:
* [[Alveolar]] [[RMS]] comprises of:
**Fibrovascular septae lined with densely packed ovoid to round tumor cells separated by pseudo-alveolar spaces (vaguely resemble pulmonary alveoli)
**Fibrovascular septae lined with densely packed [[ovoid]] to round [[Tumor cell|tumor cells]] separated by pseudo-[[alveolar]] spaces (vaguely resemble [[pulmonary alveoli]])
**"Loosely adherent" rhabdomyoblasts shed into these pseudo-alveolar spaces
**"Loosely adherent" rhabdomyoblasts shed into these pseudo-[[alveolar]] spaces
*Anaplastic RMS comprises of:
*[[Anaplastic]] [[RMS]] comprises of:
**Large hyperchromatic nuclei with atypical bizarre mitotic figures
**Large hyperchromatic [[nuclei]] with atypical bizarre [[mitotic]] figures
**Nuclear size is threefold larger than that of adjacent "typical" tumor cells
**[[Nuclear]] size is threefold larger than that of adjacent "[[Typical set|typical]]" [[Tumor cell|tumor cells]]
|Positive for:
|Positive for:
*Actin
*[[Actin]]
*Myosin
*[[Myosin]]
*Desmin
*[[Desmin]]
*Myoglobin
*[[Myoglobin]]
*Z-band protein
*Z-band [[protein]]
*Myogenic differentiation 1 (MyoD1)  
*[[Myogenic]] [[differentiation]] 1 (MyoD1)  
Maybe positive for:
Maybe positive for:
* Cytokeratin
* [[Cytokeratin]]
* Synaptophysin
* [[Synaptophysin]]
* Neuron-specific enolase
* [[Neuron-specific enolase]]
* S-100 protein
* [[S-100 protein]]
|
|
* Malignant soft tissue tumor
* [[Malignant]] [[soft tissue]] [[tumor]]
* Metastatic
* [[Metastatic]]
|
|
*In utero radiation exposure
*[[In utero]] [[radiation exposure]]
*Accelerated in utero growth
*Accelerated [[in utero]] [[growth]]
*Low socioeconomic status
*Low socioeconomic status
*Antibiotics receival soon after birth
*[[Antibiotics]] receival soon after [[birth]]
*Recreational drugs usage during pregnancy
*[[Recreational drug use|Recreational drugs usage]] during [[pregnancy]]
*Neurofibromatosis
*[[Neurofibromatosis]]
*Li-Fraumeni syndrome
*[[Li-Fraumeni syndrome]]
*Beckwith-Wiedemann syndrome
*[[Beckwith-Wiedemann syndrome]]
*Costello syndrome  
*[[Costello syndrome]]
|
|
* Head and neck (orbit, parameningeal sites including middle ear, nasal cavity, paranasal sinuses, nasopharynx, infratemporal fossa, scalp, parotid gland, oral cavity, pharynx, thyroid and parathyroid glands)  
* [[Head]] and [[neck]] ([[Orbit (anatomy)|orbit]], parameningeal sites including [[middle ear]], [[nasal cavity]], [[paranasal sinuses]], [[nasopharynx]], [[infratemporal fossa]], [[scalp]], [[parotid gland]], [[oral cavity]], [[pharynx]], [[thyroid]] and [[parathyroid glands]])  
* Genitourinary tract (bladder, prostate, or the male or female genital tracts)
* [[Genitourinary tract]] ([[Urinary bladder|bladder]], [[prostate]], or the [[male]] or [[female]] genital tracts)
*Sarcoma botryoides (urinary bladder wall, vagina, nasopharynx)
*[[Sarcoma botryoides]] ([[urinary bladder]] wall, [[vagina]], [[nasopharynx]])
* Extremity
* Extremity
*Trunk
*[[Trunk]]
*Chest wall
*[[Chest wall]]
*Perineal-perianal region
*[[Perineal]]-perianal region
*Biliary tract
*[[Biliary tract]]
*Liver
*[[Liver]]
*Brain
*[[Brain]]
*Trachea
*[[Trachea]]
*Heart
*[[Heart]]
*Breast
*[[Breast]]
*Ovary
*[[Ovary]]
|
|
*Proptosis  
*[[Proptosis]]
*Ophthalmoplegia
*[[Ophthalmoplegia]]
*Nasal, aural, or sinus obstruction with or without a mucopurulent or sanguineous discharge
*[[Nasal]], [[Aural atresia|aural]], or [[sinus]] [[obstruction]] with or without a [[mucopurulent]] or sanguineous [[discharge]]
*Localized, painless enlarging mass
*[[Localized disease|Localized]], painless enlarging [[mass]]
*Hematuria
*[[Hematuria]]
*Large pelvic mass
*Large [[Pelvic masses|pelvic mass]]
*Urinary obstruction
*[[Urinary obstruction]]
*Urinary frequency
*[[Urinary frequency]]
*Constipation
*[[Constipation]]
*Mucosanguineous vaginal discharge
*Mucosanguineous [[vaginal discharge]]
*Polypoid mass protruding from vagina
*Polypoid [[mass]] protruding from [[vagina]]
*Scrotal or inguinal enlargement (paratesticular tumors)
*[[Scrotal mass|Scrotal]] or [[inguinal]] [[Enlargement of organs|enlargement]] (paratesticular [[tumors]])
|Histology of RMS similar to that of other childhood small round blue cell tumors involving bone and soft tissue such as:
|[[Histology]] of [[RMS]] similar to that of other [[childhood]] small round blue [[cell]] [[tumors]] involving [[bone]] and [[soft tissue]] such as:
* Lymphoma
* [[Lymphoma]]
* Small cell osteosarcoma
* [[Small cell osteosarcoma]]
* Mesenchymal chondrosarcoma
* [[Mesenchymal]] [[chondrosarcoma]]
* Ewing sarcoma family of tumors (EFT)
* [[Ewing sarcoma]] family of [[tumors]] (EFT)
|-
|-
|[[Liposarcoma|'''Liposarcoma''']]<ref>{{Cite journal
| style="background:#DCDCDC;" align="center" + |[[Liposarcoma|'''Liposarcoma''']]<ref>{{Cite journal
  | author = [[Khin Thway]], [[Rashpal Flora]], [[Chirag Shah]], [[David Olmos]] & [[Cyril Fisher]]
  | author = [[Khin Thway]], [[Rashpal Flora]], [[Chirag Shah]], [[David Olmos]] & [[Cyril Fisher]]
  | title = Diagnostic utility of p16, CDK4, and MDM2 as an immunohistochemical panel in distinguishing well-differentiated and dedifferentiated liposarcomas from other adipocytic tumors
  | title = Diagnostic utility of p16, CDK4, and MDM2 as an immunohistochemical panel in distinguishing well-differentiated and dedifferentiated liposarcomas from other adipocytic tumors
Line 1,142: Line 1,138:
  | pmid = 192432
  | pmid = 192432
}}</ref>
}}</ref>
|Atypical lipomatous tumor/well differentiated liposarcoma and dedifferentiated liposarcoma are associated with:
|Atypical [[Lipomatous neoplasm|lipomatous tumor]]/well differentiated [[liposarcoma]] and dedifferentiated [[liposarcoma]] are associated with:
* Presence of a large/giant marker chromosome and/or ring chromosomes at 12q13-15 region
* Presence of a large/giant [[marker]] [[chromosome]] and/or [[Ring chromosome|ring chromosomes]] at 12q13-15 region
* Amplification of this chromosome region rich in [[Protooncogene|protooncogenes]], including ''CHOP'', ''CDK4'', ''MDM2'', ''HMGI-C'', ''GLI'', ''SAS'', ''OS1'', HMGA2 and  ''OS9''
* [[Amplification]] of this [[chromosome]] region rich in [[Protooncogene|protooncogenes]], including ''[[CHOP]]'', ''[[Cyclin-dependent kinase 4|CDK4]]'', ''[[MDM2]]'', ''HMGI-C'', ''[[GLI1|GLI]]'', ''[[SASS6|SAS]]'', ''OS1'', [[HMGA2]] and  ''[[OS9 (gene)|OS9]]''
Myxoid liposarcoma is associated with:
Myxoid [[liposarcoma]] is associated with:
*t(12:16)(q13;p11) - CHOP(DDIT3) / FUS or t(12;22)(q13;q22) - CHOP(DDIT3) / EWS
*t(12:16)(q13;p11) - [[CHOP]]([[DDIT3 gene|DDIT3]]) / [[FUS]] or t(12;22)(q13;q22) - [[CHOP]]([[DDIT3 gene|DDIT3]]) / [[EWSR1 gene|EWS]]
Pleomorphic liposarcoma is associated with:
[[Pleomorphic]] [[liposarcoma]] is associated with:
*Complex karyotypic aberrations
*[[Complex (chemistry)|Complex]] [[Karyotype|karyotypic]] aberrations
|'''Well-differentiated liposarcoma''':
|'''Well-differentiated liposarcoma''':
* '''Sclerosing liposarcoma ('''distinctive [[stromal]] cells distributed across the tissue, associated with lipoblasts filled with multiple [[vacuoles]], and [[collagenous]] background of fibrillary appearance)
* '''Sclerosing [[liposarcoma]] ('''distinctive [[stromal]] [[Cells (biology)|cells]] distributed across the [[Tissue (biology)|tissue]], associated with lipoblasts filled with multiple [[vacuoles]], and [[collagenous]] background of fibrillary appearance)
* '''Adipocytic liposarcoma''' ([[adipocytes]] with different cell sizes, hyperchromasia, and nuclear atypia. [[Fibrous]] septa containing hyperchromatic  [[stromal cells]]<nowiki/>surrounding [[adipocytes]])
* '''[[Adipocyte|Adipocytic]] [[liposarcoma]]''' ([[adipocytes]] with different [[Cell (biology)|cell]] sizes, hyperchromasia, and [[nuclear]] atypia. [[Fibrous]] [[septa]] containing hyperchromatic  [[stromal cells]]<nowiki/>surrounding [[adipocytes]])
* '''Inflammatory liposarcoma''' (heavy chronic inflammatory infiltrate composed of different lympho-plasmacytic aggregates)
* '''[[Inflammatory]] [[liposarcoma]]''' (heavy [[Chronic (medical)|chronic]] [[inflammatory]] infiltrate composed of different lympho-plasmacytic aggregates)
* '''Spindle cell lipocarcinoma''' (proliferation of neural-like [[spindle cells]] organized in a fibrous structure containing lipoblasts)
* '''[[Spindle cells|Spindle cell]] [[liposarcoma]]''' ([[proliferation]] of [[neural]]-like [[spindle cells]] organized in a [[fibrous]] [[Structure factor|structure]] containing lipoblasts)
'''De-differentiated liposarcoma''':
'''De-differentiated [[liposarcoma]]''':
* '''Myxoid liposarcoma''' ( non-homogenous appearance with cystic and solid components)
* '''Myxoid [[liposarcoma]]''' ( non-homogenous [[appearance]] with [[cystic]] and [[solid]] components)
* '''Round Cell liposarcoma''' ( small, round, or spindle cells with sparse eosinophilic and granular cytoplasm and large nuclei,scattered lipoblasts and areas of necrosis)
* '''Round [[cell]] [[liposarcoma]]''' ( small, round, or [[spindle cells]] with sparse [[Eosinophilia|eosinophilic]] and [[Granular cell|granular]] [[cytoplasm]] and large [[nuclei]],scattered lipoblasts and [[Area|areas]] of [[necrosis]])
* '''Pleiomorphic liposarcoma''' (pleomorphic cells with enlarged round to bizarre nuclei)
* '''[[Pleiomorphic]] [[liposarcoma]]''' ([[pleiomorphic]] [[Cells (biology)|cells]] with enlarged round to bizarre [[nuclei]])
|Atypical lipomatous tumor/well differentiated liposarcoma is positive for:
|Atypical [[Lipomatous neoplasm|lipomatous tumor]]/well differentiated [[liposarcoma]] is positive for:
* MDM2  
* [[MDM2]]
* CDK4
* [[Cyclin-dependent kinase 4|CDK4]]
* p16  
* [[p16]]
* S100 (stains adipocytes and lipoblasts)
* [[S100A1|S100]] ([[Stain|stains]] [[adipocytes]] and lipoblasts)
|
|
* Round cell liposarcoma metstasizes to retroperitoneum, pleural cavity, soft tissue, or pelvis and lungs (poor prognosis)
* [[Round cell carcinoma|Round cell]] [[liposarcoma]] metstasizes to [[retroperitoneum]], [[pleural cavity]], [[soft tissue]], or [[pelvis]] and [[lungs]] (poor [[prognosis]])
|
|
* Chemical carcinogens
* [[Chemical]] [[carcinogens]]
** Phenoxyacetic herbicides
** Phenoxyacetic [[herbicides]]
** Chlorophenols
** Chlorophenols
** Dioxin contaminations
** [[Dioxin]] [[Contamination|contaminations]]
** [[Arsenic]]
** [[Arsenic]]
** [[Thorium dioxide]] ([[Thorotrast]])
** [[Thorium dioxide]] ([[Thorotrast]])
* Radiation (dose of 50 GY)
* [[Radiation]] ([[dose]] of 50 GY)
* Immunodeficiency (regional acquired [[immunodeficiency]])
* [[Immunodeficiency]] (regional [[acquired]] [[immunodeficiency]])
* Genetic susceptibility
* [[Genetic]] susceptibility
*** [[Li-Fraumeni syndrome]]
*** [[Li-Fraumeni syndrome]]
*** [[Neurofibromatosis]] (NF1; von Recklinghausen disease)
*** [[Neurofibromatosis]] ([[NF1]]; [[von Recklinghausen disease]])
*** [[Gardner syndrome]] (Familial adenomatous polyposis)
*** [[Gardner syndrome]] ([[Familial adenomatous polyposis]])
*** [[Retinoblastoma]]
*** [[Retinoblastoma]]
*** [[Werner syndrome]]
*** [[Werner syndrome]]
*** [[Basal cell carcinoma|Nevoid basal cell carcinoma]] (Gorlin syndrome)
*** [[Basal cell carcinoma|Nevoid basal cell carcinoma]] ([[Gorlin syndrome]])
** [[Viral infection|Viral infections]]
** [[Viral infection|Viral infections]]
|
|
* Retroperitoneum
* [[Retroperitoneum]]
* Esophagus
* [[Esophagus]]
* Bowel
* [[Bowel]]
* Mediastinum
* [[Mediastinum]]
|
|
* '''Retroperitoneal liposarcoma''' maybe asymptomatic or causes:
* '''[[Retroperitoneal]] [[liposarcoma]]''' maybe [[asymptomatic]] or [[causes]]:
** [[Weight loss]]
** [[Weight loss]]
** [[Abdominal pain]]
** [[Abdominal pain]]
**[[Oliguria]]
**[[Oliguria]]
**[[renal failure]] (due to ureters or kidneys' compression)
**[[renal failure]] (due to [[ureters]] or [[kidneys]]' compression)
** Palpable abdominal [[mass]]
** [[Palpable]] [[abdominal]] [[mass]]
** [[Abdominal tenderness]]
** [[Abdominal tenderness]]
** [[Abdominal distention]]
** [[Abdominal distention]]
* '''Esophageal liposarcoma''' may cause:
* '''[[Esophageal]] [[liposarcoma]]''' may [[Causes|cause]]:
**[[Dysphagia]]
**[[Dysphagia]]
**[[Vomiting]]
**[[Vomiting]]
Line 1,202: Line 1,198:
**[[Gastrointestinal bleeding]]
**[[Gastrointestinal bleeding]]
**[[Hoarseness]]
**[[Hoarseness]]
* '''Bowel liposarcoma''' may cause:
* '''[[Bowel]] [[liposarcoma]]''' may cause:
**Gastrointestinal [[bleeding]]  
**[[Gastrointestinal tract|Gastrointestinal]] [[bleeding]]  
* '''Mediastinal liposarcoma''' may cause:
* '''[[Mediastinal]] [[liposarcoma]]''' may [[Causes|cause]]:
**[[Dyspnea]]
**[[Dyspnea]]
**[[Cough]]
**[[Cough]]
Line 1,211: Line 1,207:
|_
|_
|-
|-
|[[Leiomyosarcoma|'''Leiomyosarcoma''']]<ref>{{Cite journal
| style="background:#DCDCDC;" align="center" + |[[Leiomyosarcoma|'''Leiomyosarcoma''']]<ref>{{Cite journal
  | author = [[Laura M. 3rd Arnold]], [[Sudeep D. Burman]] & [[Albert H. O-Yurvati]]
  | author = [[Laura M. 3rd Arnold]], [[Sudeep D. Burman]] & [[Albert H. O-Yurvati]]
  | title = Diagnosis and management of primary pulmonary leiomyosarcoma
  | title = Diagnosis and management of primary pulmonary leiomyosarcoma
Line 1,233: Line 1,229:
}}</ref><ref name="LaunonenVierimaa2001">{{cite journal|last1=Launonen|first1=V.|last2=Vierimaa|first2=O.|last3=Kiuru|first3=M.|last4=Isola|first4=J.|last5=Roth|first5=S.|last6=Pukkala|first6=E.|last7=Sistonen|first7=P.|last8=Herva|first8=R.|last9=Aaltonen|first9=L. A.|title=Inherited susceptibility to uterine leiomyomas and renal cell cancer|journal=Proceedings of the National Academy of Sciences|volume=98|issue=6|year=2001|pages=3387–3392|issn=0027-8424|doi=10.1073/pnas.051633798}}</ref><ref name="McClainLeach1995">{{cite journal|last1=McClain|first1=Kenneth L.|last2=Leach|first2=Charles T.|last3=Jenson|first3=Hal B.|last4=Joshi|first4=Vijay V.|last5=Pollock|first5=Brad H.|last6=Parmley|first6=Richard T.|last7=DiCarlo|first7=Frederick J.|last8=Chadwick|first8=Ellen Gould|last9=Murphy|first9=Sharon B.|title=Association of Epstein–Barr Virus with Leiomyosarcomas in Young People with AIDS|journal=New England Journal of Medicine|volume=332|issue=1|year=1995|pages=12–18|issn=0028-4793|doi=10.1056/NEJM199501053320103}}</ref><ref name="FangMatsumoto2004">{{cite journal|last1=Fang|first1=Zhiwei|last2=Matsumoto|first2=Seiichi|last3=Ae|first3=Keisuke|last4=Kawaguchi|first4=Noriyoshi|last5=Yoshikawa|first5=Hideki|last6=Ueda|first6=Takafumi|last7=Ishii|first7=Takeshi|last8=Araki|first8=Nobuhito|last9=Kito|first9=Masashi|title=Postradiation soft tissue sarcoma: a multiinstitutional analysis of 14 cases in Japan|journal=Journal of Orthopaedic Science|volume=9|issue=3|year=2004|pages=242–246|issn=09492658|doi=10.1007/s00776-004-0768-5}}</ref><ref name="FuturiDonohoe2014">{{cite journal|last1=Futuri|first1=S.|last2=Donohoe|first2=K.|last3=Spaccavento|first3=C.|last4=Yudelman|first4=I.|title=Rectal leiomyosarcoma: a rare and long-term complication of radiation therapy|journal=Case Reports|volume=2014|issue=oct14 1|year=2014|pages=bcr2014205240–bcr2014205240|issn=1757-790X|doi=10.1136/bcr-2014-205240}}</ref><ref name="LynchDeters2003">{{cite journal|last1=Lynch|first1=Henry T.|last2=Deters|first2=Carolyn A.|last3=Hogg|first3=David|last4=Lynch|first4=Jane F.|last5=Kinarsky|first5=Yulia|last6=Gatalica|first6=Zoran|title=Familial sarcoma|journal=Cancer|volume=98|issue=9|year=2003|pages=1947–1957|issn=0008-543X|doi=10.1002/cncr.11743}}</ref><ref name="YuTucker2009">{{cite journal|last1=Yu|first1=C.-L.|last2=Tucker|first2=M. A.|last3=Abramson|first3=D. H.|last4=Furukawa|first4=K.|last5=Seddon|first5=J. M.|last6=Stovall|first6=M.|last7=Fraumeni|first7=J. F.|last8=Kleinerman|first8=R. A.|title=Cause-Specific Mortality in Long-Term Survivors of Retinoblastoma|journal=JNCI Journal of the National Cancer Institute|volume=101|issue=8|year=2009|pages=581–591|issn=0027-8874|doi=10.1093/jnci/djp046}}</ref>
}}</ref><ref name="LaunonenVierimaa2001">{{cite journal|last1=Launonen|first1=V.|last2=Vierimaa|first2=O.|last3=Kiuru|first3=M.|last4=Isola|first4=J.|last5=Roth|first5=S.|last6=Pukkala|first6=E.|last7=Sistonen|first7=P.|last8=Herva|first8=R.|last9=Aaltonen|first9=L. A.|title=Inherited susceptibility to uterine leiomyomas and renal cell cancer|journal=Proceedings of the National Academy of Sciences|volume=98|issue=6|year=2001|pages=3387–3392|issn=0027-8424|doi=10.1073/pnas.051633798}}</ref><ref name="McClainLeach1995">{{cite journal|last1=McClain|first1=Kenneth L.|last2=Leach|first2=Charles T.|last3=Jenson|first3=Hal B.|last4=Joshi|first4=Vijay V.|last5=Pollock|first5=Brad H.|last6=Parmley|first6=Richard T.|last7=DiCarlo|first7=Frederick J.|last8=Chadwick|first8=Ellen Gould|last9=Murphy|first9=Sharon B.|title=Association of Epstein–Barr Virus with Leiomyosarcomas in Young People with AIDS|journal=New England Journal of Medicine|volume=332|issue=1|year=1995|pages=12–18|issn=0028-4793|doi=10.1056/NEJM199501053320103}}</ref><ref name="FangMatsumoto2004">{{cite journal|last1=Fang|first1=Zhiwei|last2=Matsumoto|first2=Seiichi|last3=Ae|first3=Keisuke|last4=Kawaguchi|first4=Noriyoshi|last5=Yoshikawa|first5=Hideki|last6=Ueda|first6=Takafumi|last7=Ishii|first7=Takeshi|last8=Araki|first8=Nobuhito|last9=Kito|first9=Masashi|title=Postradiation soft tissue sarcoma: a multiinstitutional analysis of 14 cases in Japan|journal=Journal of Orthopaedic Science|volume=9|issue=3|year=2004|pages=242–246|issn=09492658|doi=10.1007/s00776-004-0768-5}}</ref><ref name="FuturiDonohoe2014">{{cite journal|last1=Futuri|first1=S.|last2=Donohoe|first2=K.|last3=Spaccavento|first3=C.|last4=Yudelman|first4=I.|title=Rectal leiomyosarcoma: a rare and long-term complication of radiation therapy|journal=Case Reports|volume=2014|issue=oct14 1|year=2014|pages=bcr2014205240–bcr2014205240|issn=1757-790X|doi=10.1136/bcr-2014-205240}}</ref><ref name="LynchDeters2003">{{cite journal|last1=Lynch|first1=Henry T.|last2=Deters|first2=Carolyn A.|last3=Hogg|first3=David|last4=Lynch|first4=Jane F.|last5=Kinarsky|first5=Yulia|last6=Gatalica|first6=Zoran|title=Familial sarcoma|journal=Cancer|volume=98|issue=9|year=2003|pages=1947–1957|issn=0008-543X|doi=10.1002/cncr.11743}}</ref><ref name="YuTucker2009">{{cite journal|last1=Yu|first1=C.-L.|last2=Tucker|first2=M. A.|last3=Abramson|first3=D. H.|last4=Furukawa|first4=K.|last5=Seddon|first5=J. M.|last6=Stovall|first6=M.|last7=Fraumeni|first7=J. F.|last8=Kleinerman|first8=R. A.|title=Cause-Specific Mortality in Long-Term Survivors of Retinoblastoma|journal=JNCI Journal of the National Cancer Institute|volume=101|issue=8|year=2009|pages=581–591|issn=0027-8874|doi=10.1093/jnci/djp046}}</ref>
|
|
* Loss of normal [[chromosome]] 1q (hereditary leiomyomatosis)
* Loss of normal [[chromosome]] 1q ([[hereditary]] [[leiomyomatosis]])
* [[Renal cell cancer]] (HLRCC) gene mutation
* [[Renal cell cancer]] (HLRCC) [[gene mutation]]
|
|
* Prominent cellular [[atypia]]
* Prominent [[cellular]] [[atypia]]
* Nuclear [[atypia]], including nuclear [[pleomorphism]], hyperchromatism, irregularity in [[nuclear]] membranes, high [[nuclear]] size, and prominent [[nucleoli]]
* [[Nuclear]] [[atypia]], including [[nuclear]][[pleomorphism]], hyperchromatism, irregularity in [[nuclear]] membranes, high [[nuclear]] size, and prominent [[nucleoli]]
* Abundant [[mitoses]], [[mitotic index]] higher than10 or more per 10 high-power fields
* [[Cigar]]-shaped [[nuclei]]
* Areas of [[coagulative necrosis]] (tumor cell [[necrosis]]), always accompanied by [[Cytological|cytologic]] [[atypia]] and brisk [[mitotic]] activity
* Abundant [[mitoses]], [[mitotic index]] higher than 10 or more per 10 high-power fields
* [[Area]]<nowiki/>s of [[coagulative necrosis]] ([[tumor cell]][[necrosis]])
* Palisading and extensive [[degenerative]]<nowiki/>changes in the form of hyalinization, [[calcification]], and myxoid changes
* Elongated [[Cells (biology)|cells]] with [[eosinophilic]] or occasional [[Fibrillarin|fibrillar]] [[cytoplasm]] with [[Distinctive feature|distinct]][[cell membranes]]  
|Positive for:
|Positive for:
* HHF35 (90%)
* HHF35 (90%)
* Alpha-smooth muscle actin (90%)
* Alpha-[[smooth muscle]] [[actin]] (90%)
* Vimentin
* [[Vimentin]]
* Desmin (75%)
* [[Desmin]] (75%)
* H-caldesmon  
* H-[[caldesmon]]
* PTAH (stains myofibrils)
* [[Phosphotungstic acid hematoxylin|PTAH]] ([[Stain|stains]] [[myofibrils]])
* Keratin (30%)
* [[Keratin]] (30%)
* ER (usually in uterine and female retroperitoneal tumors)
* [[ER]] (usually in [[uterine]] and [[female]] [[retroperitoneal]] [[tumors]])
* S100 (occasionally weak staining)
* [[S100A1|S100]] (occasionally weak [[staining]])
* EMA (may be focal)
* EMA (may be focal)
* CD34
* [[CD34]]
Negative for:
Negative for:
* CD117
* [[CD117]]
|
|
* Malignant
* [[Malignant]]
* Metastatic to  [[lungs]], [[liver]], [[blood vessels]], or any other [[soft tissue]] in the body
* [[Metastatic]] to  [[lungs]], [[liver]], [[blood vessels]], or any other [[soft tissue]] in the [[body]]
|
|
* Immundeficiency (HIV)
* Immundeficiency ([[HIV]])
* [[Dioxin|Digoxin]] exposure
* [[Dioxin|Digoxin]] [[Exposure assessment|exposure]]
* Human herpes virus type-8 ([[HHV-8]])
* [[HHV-8|Human herpes virus type-8 (HHV-8)]]
* [[Epstein barr virus mononucleosis|Epstein barr virus]]
* [[Epstein barr virus mononucleosis|Epstein barr virus]]
* Long term [[tamoxifen]] use
* Long term [[tamoxifen]] use
* History of pelvic radiations
* [[History and Physical examination|History]] of [[pelvic]] [[Radiation|radiations]]
* Hereditary breast carcinoma with [[BRCA1]] mutation
* [[Hereditary]] [[breast carcinoma]] with [[BRCA1]] [[mutation]]
* [[Hereditary nonpolyposis colorectal cancer|Hereditary nonpolyposis colorectal carcinoma]] with [[MSH2]] mutation
* [[Hereditary nonpolyposis colorectal cancer|Hereditary nonpolyposis colorectal carcinoma]] with [[MSH2]] [[mutation]]
* [[Li-Fraumeni syndrome]]
* [[Li-Fraumeni syndrome]]
* Malignant [[melanoma]]
* [[Malignant]] [[melanoma]]
* [[Retinoblastoma]]  
* [[Retinoblastoma]]  
|
|
* Uterus
* [[Uterus]]
* Abdomen
* [[Abdomen]]
* Esophagus
* [[Esophagus]]
* Rectum
* [[Rectum]]
* Skin / subcutis
* [[Skin]] / [[subcutis]]
* Retroperitoneum
* [[Retroperitoneum]]
* Extremities
* [[Extremities]]
* Large vessels (inferior vena cava, saphenous vein, femoral vein, pulmonary artery, femoral artery)
* Large [[vessels]] ([[inferior vena cava]], [[saphenous vein]], [[femoral vein]], [[pulmonary artery]], [[femoral artery]])
* Superficial or deep soft tissues
* [[Superficial]] or deep [[Soft tissue|soft tissues]]
* Bone
* [[Bone]]
* Breast
* [[Breast]]
* Colon
* [[Colon]]
* Epididymis
* [[Epididymis]]
* Mediastinum
* [[Mediastinum]]
* Lungs
* [[Lungs]]
|
|
*Asymptomatic  
*[[Asymptomatic]]
*[[Uterine|(uterine]] leiomyosarcoma may be associated with:
*[[Uterine|(uterine]] [[leiomyosarcoma]] may be associated with:
**Irregular [[vaginal bleeding]] (intermenstrual or [[postmenopausal]])
**Irregular [[vaginal bleeding]] (intermenstrual or [[postmenopausal]])
**New [[lump]] or a [[mass]] protruding into vagina or growing mass in [[abdomen]] or [[pelvis]]
**New [[lump]] or a [[mass]] protruding into [[vagina]] or growing [[mass]] in [[abdomen]] or [[pelvis]]
**[[Abdominal pain]]  
**[[Abdominal pain]]  
**Abdominal distension
**[[Abdominal distension]]
**[[Pelvic pain]]
**[[Pelvic pain]]
**Urinary symptoms
**[[Urinary system|Urinary]] [[symptoms]]
*Esophageal leiomyosarcoma may cause:
*[[Esophageal]] [[leiomyosarcoma]] may cause:
**Dysphagia
**[[Dysphagia]]
**[[Hematemesis]]
**[[Hematemesis]]
*[[rectal]] leiomyosarcoma may cause:
*[[rectal]] [[leiomyosarcoma]] may cause:
** Black, [[tarry stools]]
** [[Black]], [[tarry stools]]
**[[Rectal bleeding]]
**[[Rectal bleeding]]
|_
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|-
|-
|[[Neurofibroma|'''Neurofibroma''']]<ref name="RodriguezFolpe2012">{{cite journal|last1=Rodriguez|first1=Fausto J.|last2=Folpe|first2=Andrew L.|last3=Giannini|first3=Caterina|last4=Perry|first4=Arie|title=Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems|journal=Acta Neuropathologica|volume=123|issue=3|year=2012|pages=295–319|issn=0001-6322|doi=10.1007/s00401-012-0954-z}}</ref><ref name="ChoiKomurov2017">{{cite journal|last1=Choi|first1=Kwangmin|last2=Komurov|first2=Kakajan|last3=Fletcher|first3=Jonathan S.|last4=Jousma|first4=Edwin|last5=Cancelas|first5=Jose A.|last6=Wu|first6=Jianqiang|last7=Ratner|first7=Nancy|title=An inflammatory gene signature distinguishes neurofibroma Schwann cells and macrophages from cells in the normal peripheral nervous system|journal=Scientific Reports|volume=7|issue=1|year=2017|issn=2045-2322|doi=10.1038/srep43315}}</ref><ref name="LiaoBooker2018">{{cite journal|last1=Liao|first1=Chung-Ping|last2=Booker|first2=Reid C.|last3=Brosseau|first3=Jean-Philippe|last4=Chen|first4=Zhiguo|last5=Mo|first5=Juan|last6=Tchegnon|first6=Edem|last7=Wang|first7=Yong|last8=Clapp|first8=D. Wade|last9=Le|first9=Lu Q.|title=Contributions of inflammation and tumor microenvironment to neurofibroma tumorigenesis|journal=Journal of Clinical Investigation|volume=128|issue=7|year=2018|pages=2848–2861|issn=0021-9738|doi=10.1172/JCI99424}}</ref><ref name="StaserYang2010">{{cite journal|last1=Staser|first1=K.|last2=Yang|first2=F.-C.|last3=Clapp|first3=D. W.|title=Mast cells and the neurofibroma microenvironment|journal=Blood|volume=116|issue=2|year=2010|pages=157–164|issn=0006-4971|doi=10.1182/blood-2009-09-242875}}</ref><ref name="MuirNeubauer2001">{{cite journal|last1=Muir|first1=David|last2=Neubauer|first2=Debbie|last3=Lim|first3=Ingrid T.|last4=Yachnis|first4=Anthony T.|last5=Wallace|first5=Margaret R.|title=Tumorigenic Properties of Neurofibromin-Deficient Neurofibroma Schwann Cells|journal=The American Journal of Pathology|volume=158|issue=2|year=2001|pages=501–513|issn=00029440|doi=10.1016/S0002-9440(10)63992-2}}</ref><ref name="WilkinsonManson2004">{{cite journal|last1=Wilkinson|first1=Lana M.|last2=Manson|first2=David|last3=Smith|first3=Charles R.|title=Best Cases from the AFIP|journal=RadioGraphics|volume=24|issue=suppl_1|year=2004|pages=S237–S242|issn=0271-5333|doi=10.1148/rg.24si035170}}</ref><ref name="BernthalJones2013">{{cite journal|last1=Bernthal|first1=Nicholas|last2=Jones|first2=Kevin|last3=Monument|first3=Michael|last4=Liu|first4=Ting|last5=Viskochil|first5=David|last6=Randall|first6=R.|title=Lost in Translation: Ambiguity in Nerve Sheath Tumor Nomenclature and Its Resultant Treatment Effect|journal=Cancers|volume=5|issue=4|year=2013|pages=519–528|issn=2072-6694|doi=10.3390/cancers5020519}}</ref><ref name="StaserYang2010">{{cite journal|last1=Staser|first1=K.|last2=Yang|first2=F.-C.|last3=Clapp|first3=D. W.|title=Mast cells and the neurofibroma microenvironment|journal=Blood|volume=116|issue=2|year=2010|pages=157–164|issn=0006-4971|doi=10.1182/blood-2009-09-242875}}</ref><ref name="MautnerFriedrich2003">{{cite journal|last1=Mautner|first1=V. F.|last2=Friedrich|first2=R. E.|last3=von Deimling|first3=A.|last4=Hagel|first4=C.|last5=Korf|first5=B.|last6=Knöfel|first6=M. T.|last7=Wenzel|first7=R.|last8=Fünsterer|first8=C.|title=Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma|journal=Neuroradiology|volume=45|issue=9|year=2003|pages=618–625|issn=0028-3940|doi=10.1007/s00234-003-0964-6}}</ref><ref name="ShenHarper1996">{{cite journal|last1=Shen|first1=M H|last2=Harper|first2=P S|last3=Upadhyaya|first3=M|title=Molecular genetics of neurofibromatosis type 1 (NF1).|journal=Journal of Medical Genetics|volume=33|issue=1|year=1996|pages=2–17|issn=1468-6244|doi=10.1136/jmg.33.1.2}}</ref><ref name="RubinGutmann2005">{{cite journal|last1=Rubin|first1=Joshua B.|last2=Gutmann|first2=David H.|title=Neurofibromatosis type 1 — a model for nervous system tumour formation?|journal=Nature Reviews Cancer|volume=5|issue=7|year=2005|pages=557–564|issn=1474-175X|doi=10.1038/nrc1653}}</ref>
| style="background:#DCDCDC;" align="center" + |[[Neurofibroma|'''Neurofibroma''']]<ref name="RodriguezFolpe2012">{{cite journal|last1=Rodriguez|first1=Fausto J.|last2=Folpe|first2=Andrew L.|last3=Giannini|first3=Caterina|last4=Perry|first4=Arie|title=Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems|journal=Acta Neuropathologica|volume=123|issue=3|year=2012|pages=295–319|issn=0001-6322|doi=10.1007/s00401-012-0954-z}}</ref><ref name="ChoiKomurov2017">{{cite journal|last1=Choi|first1=Kwangmin|last2=Komurov|first2=Kakajan|last3=Fletcher|first3=Jonathan S.|last4=Jousma|first4=Edwin|last5=Cancelas|first5=Jose A.|last6=Wu|first6=Jianqiang|last7=Ratner|first7=Nancy|title=An inflammatory gene signature distinguishes neurofibroma Schwann cells and macrophages from cells in the normal peripheral nervous system|journal=Scientific Reports|volume=7|issue=1|year=2017|issn=2045-2322|doi=10.1038/srep43315}}</ref><ref name="LiaoBooker2018">{{cite journal|last1=Liao|first1=Chung-Ping|last2=Booker|first2=Reid C.|last3=Brosseau|first3=Jean-Philippe|last4=Chen|first4=Zhiguo|last5=Mo|first5=Juan|last6=Tchegnon|first6=Edem|last7=Wang|first7=Yong|last8=Clapp|first8=D. Wade|last9=Le|first9=Lu Q.|title=Contributions of inflammation and tumor microenvironment to neurofibroma tumorigenesis|journal=Journal of Clinical Investigation|volume=128|issue=7|year=2018|pages=2848–2861|issn=0021-9738|doi=10.1172/JCI99424}}</ref><ref name="StaserYang2010">{{cite journal|last1=Staser|first1=K.|last2=Yang|first2=F.-C.|last3=Clapp|first3=D. W.|title=Mast cells and the neurofibroma microenvironment|journal=Blood|volume=116|issue=2|year=2010|pages=157–164|issn=0006-4971|doi=10.1182/blood-2009-09-242875}}</ref><ref name="MuirNeubauer2001">{{cite journal|last1=Muir|first1=David|last2=Neubauer|first2=Debbie|last3=Lim|first3=Ingrid T.|last4=Yachnis|first4=Anthony T.|last5=Wallace|first5=Margaret R.|title=Tumorigenic Properties of Neurofibromin-Deficient Neurofibroma Schwann Cells|journal=The American Journal of Pathology|volume=158|issue=2|year=2001|pages=501–513|issn=00029440|doi=10.1016/S0002-9440(10)63992-2}}</ref><ref name="WilkinsonManson2004">{{cite journal|last1=Wilkinson|first1=Lana M.|last2=Manson|first2=David|last3=Smith|first3=Charles R.|title=Best Cases from the AFIP|journal=RadioGraphics|volume=24|issue=suppl_1|year=2004|pages=S237–S242|issn=0271-5333|doi=10.1148/rg.24si035170}}</ref><ref name="BernthalJones2013">{{cite journal|last1=Bernthal|first1=Nicholas|last2=Jones|first2=Kevin|last3=Monument|first3=Michael|last4=Liu|first4=Ting|last5=Viskochil|first5=David|last6=Randall|first6=R.|title=Lost in Translation: Ambiguity in Nerve Sheath Tumor Nomenclature and Its Resultant Treatment Effect|journal=Cancers|volume=5|issue=4|year=2013|pages=519–528|issn=2072-6694|doi=10.3390/cancers5020519}}</ref><ref name="StaserYang2010">{{cite journal|last1=Staser|first1=K.|last2=Yang|first2=F.-C.|last3=Clapp|first3=D. W.|title=Mast cells and the neurofibroma microenvironment|journal=Blood|volume=116|issue=2|year=2010|pages=157–164|issn=0006-4971|doi=10.1182/blood-2009-09-242875}}</ref><ref name="MautnerFriedrich2003">{{cite journal|last1=Mautner|first1=V. F.|last2=Friedrich|first2=R. E.|last3=von Deimling|first3=A.|last4=Hagel|first4=C.|last5=Korf|first5=B.|last6=Knöfel|first6=M. T.|last7=Wenzel|first7=R.|last8=Fünsterer|first8=C.|title=Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma|journal=Neuroradiology|volume=45|issue=9|year=2003|pages=618–625|issn=0028-3940|doi=10.1007/s00234-003-0964-6}}</ref><ref name="ShenHarper1996">{{cite journal|last1=Shen|first1=M H|last2=Harper|first2=P S|last3=Upadhyaya|first3=M|title=Molecular genetics of neurofibromatosis type 1 (NF1).|journal=Journal of Medical Genetics|volume=33|issue=1|year=1996|pages=2–17|issn=1468-6244|doi=10.1136/jmg.33.1.2}}</ref><ref name="RubinGutmann2005">{{cite journal|last1=Rubin|first1=Joshua B.|last2=Gutmann|first2=David H.|title=Neurofibromatosis type 1 — a model for nervous system tumour formation?|journal=Nature Reviews Cancer|volume=5|issue=7|year=2005|pages=557–564|issn=1474-175X|doi=10.1038/nrc1653}}</ref>
|Can be sporadic or as a part of Neurofibromatosis 1 and 2
|Can be sporadic or as a part of [[Neurofibromatosis 1]] and 2
* ''[[Neurofibromatosis type I|NF1]] gene located at chromosomal region [[CCL7|17q11.2]], codes for'' [[neurofibromin]]
* ''[[Neurofibromatosis type I|NF1]] [[gene]] located at [[chromosomal]] region [[CCL7|17q11.2]], [[Code|codes]]<nowiki/>for''[[neurofibromin]]
* Functional part of neurofibromin GAP (or GTPase-activating protein) accelerates the conversion of the active GTP-bound RAS to its inactive GDP-bound form
* Functional part of [[neurofibromin]] GAP (or [[GTPase-activating proteins|GTPase-activating protein]]) accelerates the [[Conversion (logic)|conversion]]<nowiki/>of the active [[GTP-binding protein|GTP]]-bound [[RAS]] to its inactive GDP-[[Bound state|bound]] form
* Loss of ''[[RAS]]'' control leads to increased activity of other signaling pathways including ''[[C-Raf|RAF]]'', ''[[Extracellular signal-regulated kinases|ERK1/2]]'', ''[[Phosphoinositide 3-kinase|PI3K]]'', ''PAK, MAPK, SCF/c-kit'' and ''[[Mammalian target of rapamycin|mTOR-S6 kinase]]''
* Loss of ''[[RAS]]'' [[control]]<nowiki/>leads to increased [[Activity (chemistry)|activity]] of other [[Signaling pathway|signaling pathways]]<nowiki/>including ''[[C-Raf|RAF]]'', ''[[Extracellular signal-regulated kinases|ERK1/2]]'', ''[[Phosphoinositide 3-kinase|PI3K]]'', ''[[PAK1|PAK]], [[MAPK]], [[SCF-complex|SCF]]/[[c-kit]]'' and ''[[Mammalian target of rapamycin|mTOR-S6 kinase]]''
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|
* Made of nerve fibers, schwann cells (cells that cover the nerve fibers), blood vessels, inflammatory white blood cells (mast cells), and connective tissue (fibroblasts and loose material called extra cellular matrix)
* Uniphasic, low to moderate cellularity
* No peripheral perineural [[capsule]]
* [[Random]] [[pattern]], only [[rare]] palisading
* No well formed verocy bodies
* Hypocellular with abundant [[mucinous]]/myxoid [[matrix]] without hypercellular [[Area|areas]]
* Frequent [[mast cells]]
* Contains [[neural]] [[fibroblasts]] and fibrillary or shredded carrot [[collagen]]
* [[Random]] [[proliferation]] of [[Schwann cells]]<nowiki/>and scattered admixed [[axons]]
* No [[Nevi|nevoid cells]]
* No [[epithelial]] component
* [[Diffuse]] [[growth]] [[pattern]]
* Scant [[cytoplasm]]
* Wavy [[spindle cells]] with buckled [[nuclei]]
* Pseudomeissnerian [[Body|bodies]] representing specific [[differentiation]] may be present
* Lacks storiform [[pattern]]


* [[Spindle cell|Spindle cells]] with wavy [[nuclei]] without pleomorphism
* [[Neurofibroma]] with [[degenerative]] [[atypia]]("ancient [[Change detection|change]]") has following [[microscopic]] [[Features (pattern recognition)|features]]:
* Intermixed with wire-like [[collagen]]
** [[Localized disease|Localized]] [[Cells (biology)|cells]] with large [[pleomorphic]][[nuclei]], [[cytoplasmic]] [[nuclear]] [[inclusions]], smudgy [[chromatin]], and inconspicuous [[nuclei]]
* Moderate increase of cellularity vis-a-vis normal [[dermis]]
** Absent or very low [[Mitotic|mitotic activity]]
* [[Mast cells]]
** Low to moderate cellularity
|Positive for:
|Positive for:
* S100
* [[S100A12|S100]] (weaker)
* CD34 (focal)
* [[SOX10]]
* Factor XIIIa (focal)
* [[Neurofilament]] (and Bielshowsky)
* [[GFAP]]
* [[CD34]] (stronger)
* Factor XIIIa
* [[Calretinin]] (focal)
* MBP ([[myelin]]-[[Basic (chemistry)|basic]] [[protein]])
Negative for:
Negative for:
* EMA (except in plexiform neurofibromas)
* EMA (except in plexiform [[Neurofibroma|neurofibromas]])
|
* [[Cutaneous]] [[Neurofibroma|neurofibromas]] are always [[benign]]
* [[Diffuse]] [[Neurofibroma|neurofibromas]] can become [[malignant]]
|
|
* Cutaneous neurofibromas are always benign
* [[Neurofibromatosis type I|Neurofibromatosis 1]]
* Diffuse neurofibromas can become malignant
* [[Neurofibromatosis 2]] (multiple [[Neurofibroma|neurofibromas]], [[meningiomas]] of the [[brain]] or [[spinal cord]], and [[ependymomas]] of the [[spinal cord]])
|
|
* Neurofibromatosis 1
* Anywhere in [[Human body|body]]
* Neurofibromatosis 2 (multiple neurofibromas, meningiomas of the brain or spinal cord, and ependymomas of the spinal cord)
* [[Scalp]] ([[diffuse]] [[neurofibroma]])
|
|
* Anywhere in body
* Soft [[Mass|masses]]/[[Bumps on skin|bumps on or under skin]] ([[internal]] or [[superficial]])
* Scalp (diffuse neurofibroma)
* [[Transient]] [[itching]] ([[mast cells]]<nowiki/>release [[histamine]])
* [[Transient]] [[pain]]
* [[Numbness]] and [[tingling]] in the affected [[area]]
* Severe [[bleeding]] (sign of [[tumor]][[growth]])
* [[Physical therapy|Physical]] disfiguration
* [[Cognitive]] [[disability]]
* [[Stinging in the eye|Stinging]]
* [[Neurological]] [[Deficits in Attention, Motor control and Perception|deficits]]
* [[Change detection|Changes]] in [[Movement disorder|movement]]([[clumsiness]] in [[hands]], trouble [[walking]])
* [[Bowel]] [[incontinence]]
* [[Scoliosis]] (an [[abnormal]] [[Curvature of spine|curvature of the spine]], if the [[tumor]] creates [[muscular]] [[imbalance]] or erodes [[bones]] of the [[spine]])
* Following [[symptoms]] may occur with [[genitourinary tract]]<nowiki/>involvement (rarely):
 
** [[Urinary tract infection]] (most common [[clinical]]<nowiki/>manifestation)
** [[Urinary retention]]
** [[Urinary frequency]]
** [[Urgency]]
** [[Hematuria]]
** Pelvic mass
** [[Hydronephrosis)|Hydronephrosis]]
** [[Urinary incontinence]](decreased [[Urinary bladder|bladder]] capacity or [[compliance]])
** [[Appearance|Appears]] as a focal [[mass]] or [[diffuse]] [[Urinary bladder|bladder]] wall thickening in case of a [[plexiform neurofibroma]]
|
|
* Soft [[Mass|masses]]/bumps on or under skin (internal or superficial)
* [[Nerve]] often not identified, incorporates [[nerve]], [[axons]]<nowiki/>often present in [[lesion]]
* Transient [[itching]] (mast cells release histamine)
* Seldom [[cystic]]
* Transient [[pain]]
* Frequently multiple
* Numbness and tingling in the affected area
* Widespread [[soft tissue]][[Infiltration (medical)|infiltration]]
* Severe bleeding (sign of tumor growth)
* Tends to displace [[Adnexal and skin appendage neoplasms|adnexa]]
* Physical disfiguration
* <2cm in [[diameter]]
* Cognitive disability
* [[Lack (manque)|Lacks]] [[Distinctive feature|distinct]] [[Lobule|lobulation]]
* Stinging
* [[Lack (manque)|Lacks]] [[fat]]
* Neurological deficits
* Affects [[Individual growth|individuals]] between 20-40 [[Year|years]] of [[age]]
* Changes in movement (clumsiness in the hands, trouble walking
* Men and women are [[Equalism|equally]]<nowiki/>affected
* Changes in bowel or bladder function (a sense of urgency when urinating, bowel or bladder incontinence)
* [[Plexiform neurofibroma]] are thought to be [[Congenital disorder|congenital]] and occur earlier in [[life]]
* Scoliosis (an abnormal curvature of the spine, if the tumor creates muscular imbalance or erodes bones of the spine)
|Types of neurofibromas in NF1:
* Diffuse neurofibromas
* Cutaneous or Dermal neurofibromas
* Intramuscular neurofibromas
* Plexiform neurofibromas
* Intraneural
|-
|-
|[[Nodular fasciitis|'''Nodular fasciitis''']]<ref name="pmid18725954">{{cite journal| author=Sailon AM, Cappuccino G, Hameed M, Fleegler EJ| title=Nodular fasciitis of the hand over the metacarpophalangeal joint: a case report. | journal=Eplasty | year= 2008 | volume= 8 | issue=  | pages= e38 | pmid=18725954 | doi= | pmc=2491338 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18725954  }} </ref><ref name="EmoriShimizu2018">{{cite journal|last1=Emori|first1=M|last2=Shimizu|first2=J|last3=Murahashi|first3=Y|last4=Mizushima|first4=E|last5=Sugita|first5=S|last6=Hasegawa|first6=T|last7=Yamashita|first7=T|title=Nodular fasciitis involving the palm|journal=The Annals of The Royal College of Surgeons of England|volume=100|issue=5|year=2018|pages=e128–e131|issn=0035-8843|doi=10.1308/rcsann.2018.0061}}</ref><ref name="KubotaOkada2012">{{cite journal|last1=Kubota|first1=Kensuke|last2=Okada|first2=Seiji|last3=Maeda|first3=Takeshi|last4=Matsumoto|first4=Yoshihiro|last5=Sakamoto|first5=Akio|last6=Harimaya|first6=Katsumi|last7=Saiwai|first7=Hirokazu|last8=Kumamaru|first8=Hiromi|last9=Oda|first9=Yoshinao|last10=Iwamoto|first10=Yukihide|title=Extradural Nodular Fasciitis Arising in the Spinal Canal|journal=Spine|volume=37|issue=2|year=2012|pages=E133–E137|issn=0362-2436|doi=10.1097/BRS.0b013e318224568a}}</ref><ref name="pmid15716235">{{cite journal| author=Plaza JA, Mayerson J, Wakely PE| title=Nodular fasciitis of the hand: a potential diagnostic pitfall in fine-needle aspiration cytopathology. | journal=Am J Clin Pathol | year= 2005 | volume= 123 | issue= 3 | pages= 388-93 | pmid=15716235 | doi=10.1309/PWD0-HB51-1L3V-R56W | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15716235  }} </ref><ref name="pmid20975584">{{cite journal| author=Wagner LM, Gelfand MJ, Laor T, Ryckman FC, Al-Ghawi H, Bove KE| title=A welcome surprise: nodular fasciitis presenting as soft tissue sarcoma. | journal=J Pediatr Hematol Oncol | year= 2011 | volume= 33 | issue= 4 | pages= 316-9 | pmid=20975584 | doi=10.1097/MPH.0b013e3181e88649 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20975584  }} </ref><ref name="pmid19655607">{{cite journal| author=Grobmyer SR, Knapik JA, Foss RM, Copeland EM, Hochwald SN| title=Nodular fasciitis: differential considerations and current management strategies. | journal=Am Surg | year= 2009 | volume= 75 | issue= 7 | pages= 610-4 | pmid=19655607 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19655607  }} </ref><ref name="pmid9528235">{{cite journal| author=Yamamoto H, Yamada N, Yamada I, Hashikawa K, Kagimoto K, Ookubo M et al.| title=[A case of nodular fasciitis of the chest wall]. | journal=Kyobu Geka | year= 1998 | volume= 51 | issue= 3 | pages= 246-9 | pmid=9528235 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9528235  }} </ref><ref name="pmid18330617">{{cite journal| author=Chiarini L, Lo Russo L, Figurelli S, Procacci P, Rubini C, Lo Muzio L| title=Nodular fasciitis of the face: aesthetic considerations. | journal=Aesthetic Plast Surg | year= 2008 | volume= 32 | issue= 3 | pages= 560-2 | pmid=18330617 | doi=10.1007/s00266-008-9136-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18330617  }} </ref><ref name="pmid23769422">{{cite journal| author=Oliveira AM, Chou MM| title=USP6-induced neoplasms: the biologic spectrum of aneurysmal bone cyst and nodular fasciitis. | journal=Hum Pathol | year= 2014 | volume= 45 | issue= 1 | pages= 1-11 | pmid=23769422 | doi=10.1016/j.humpath.2013.03.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23769422  }} </ref><ref name="pmid21826056">{{cite journal| author=Erickson-Johnson MR, Chou MM, Evers BR, Roth CW, Seys AR, Jin L et al.| title=Nodular fasciitis: a novel model of transient neoplasia induced by MYH9-USP6 gene fusion. | journal=Lab Invest | year= 2011 | volume= 91 | issue= 10 | pages= 1427-33 | pmid=21826056 | doi=10.1038/labinvest.2011.118 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21826056  }} </ref>
| style="background:#DCDCDC;" align="center" + |[[Nodular fasciitis|'''Nodular fasciitis''']]<ref name="pmid18725954">{{cite journal| author=Sailon AM, Cappuccino G, Hameed M, Fleegler EJ| title=Nodular fasciitis of the hand over the metacarpophalangeal joint: a case report. | journal=Eplasty | year= 2008 | volume= 8 | issue=  | pages= e38 | pmid=18725954 | doi= | pmc=2491338 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18725954  }} </ref><ref name="EmoriShimizu2018">{{cite journal|last1=Emori|first1=M|last2=Shimizu|first2=J|last3=Murahashi|first3=Y|last4=Mizushima|first4=E|last5=Sugita|first5=S|last6=Hasegawa|first6=T|last7=Yamashita|first7=T|title=Nodular fasciitis involving the palm|journal=The Annals of The Royal College of Surgeons of England|volume=100|issue=5|year=2018|pages=e128–e131|issn=0035-8843|doi=10.1308/rcsann.2018.0061}}</ref><ref name="KubotaOkada2012">{{cite journal|last1=Kubota|first1=Kensuke|last2=Okada|first2=Seiji|last3=Maeda|first3=Takeshi|last4=Matsumoto|first4=Yoshihiro|last5=Sakamoto|first5=Akio|last6=Harimaya|first6=Katsumi|last7=Saiwai|first7=Hirokazu|last8=Kumamaru|first8=Hiromi|last9=Oda|first9=Yoshinao|last10=Iwamoto|first10=Yukihide|title=Extradural Nodular Fasciitis Arising in the Spinal Canal|journal=Spine|volume=37|issue=2|year=2012|pages=E133–E137|issn=0362-2436|doi=10.1097/BRS.0b013e318224568a}}</ref><ref name="pmid15716235">{{cite journal| author=Plaza JA, Mayerson J, Wakely PE| title=Nodular fasciitis of the hand: a potential diagnostic pitfall in fine-needle aspiration cytopathology. | journal=Am J Clin Pathol | year= 2005 | volume= 123 | issue= 3 | pages= 388-93 | pmid=15716235 | doi=10.1309/PWD0-HB51-1L3V-R56W | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15716235  }} </ref><ref name="pmid20975584">{{cite journal| author=Wagner LM, Gelfand MJ, Laor T, Ryckman FC, Al-Ghawi H, Bove KE| title=A welcome surprise: nodular fasciitis presenting as soft tissue sarcoma. | journal=J Pediatr Hematol Oncol | year= 2011 | volume= 33 | issue= 4 | pages= 316-9 | pmid=20975584 | doi=10.1097/MPH.0b013e3181e88649 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20975584  }} </ref><ref name="pmid19655607">{{cite journal| author=Grobmyer SR, Knapik JA, Foss RM, Copeland EM, Hochwald SN| title=Nodular fasciitis: differential considerations and current management strategies. | journal=Am Surg | year= 2009 | volume= 75 | issue= 7 | pages= 610-4 | pmid=19655607 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19655607  }} </ref><ref name="pmid9528235">{{cite journal| author=Yamamoto H, Yamada N, Yamada I, Hashikawa K, Kagimoto K, Ookubo M et al.| title=[A case of nodular fasciitis of the chest wall]. | journal=Kyobu Geka | year= 1998 | volume= 51 | issue= 3 | pages= 246-9 | pmid=9528235 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9528235  }} </ref><ref name="pmid18330617">{{cite journal| author=Chiarini L, Lo Russo L, Figurelli S, Procacci P, Rubini C, Lo Muzio L| title=Nodular fasciitis of the face: aesthetic considerations. | journal=Aesthetic Plast Surg | year= 2008 | volume= 32 | issue= 3 | pages= 560-2 | pmid=18330617 | doi=10.1007/s00266-008-9136-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18330617  }} </ref><ref name="pmid23769422">{{cite journal| author=Oliveira AM, Chou MM| title=USP6-induced neoplasms: the biologic spectrum of aneurysmal bone cyst and nodular fasciitis. | journal=Hum Pathol | year= 2014 | volume= 45 | issue= 1 | pages= 1-11 | pmid=23769422 | doi=10.1016/j.humpath.2013.03.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23769422  }} </ref><ref name="pmid21826056">{{cite journal| author=Erickson-Johnson MR, Chou MM, Evers BR, Roth CW, Seys AR, Jin L et al.| title=Nodular fasciitis: a novel model of transient neoplasia induced by MYH9-USP6 gene fusion. | journal=Lab Invest | year= 2011 | volume= 91 | issue= 10 | pages= 1427-33 | pmid=21826056 | doi=10.1038/labinvest.2011.118 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21826056  }} </ref>
|
|
* Balanced translocation t(17;22)(p13;q13) resulting in MYH9-USP6 gene fusion
* [[Balanced translocation]] t(17;22)(p13;q13) resulting in [[MYH9]]-[[USP6]] [[gene fusion]]
|
|
* Histologically vast array of patterns
* [[Histologically]] vast array of patterns
* Fibroblasts, spindle-shaped cells found in connective tissue
* [[Fibroblasts]], [[spindle]]-shaped [[Cells (biology)|cells]] found in [[connective tissue]]
* Myofibroblasts, large cells with an appearance between fibroblasts and smooth muscle tissue
* [[Myofibroblasts]], large [[Cells (biology)|cells]] with an [[appearance]] between [[fibroblasts]] and [[smooth muscle]] [[tissue]]
* Sub-membranous "tram track" pattern characteristic of myofibroblasts on immunostaining
* Sub-membranous "tram track" [[pattern]] characteristic of [[myofibroblasts]] on [[immunostaining]]
* Short C or S-shaped fascicles or cartwheel formations
* Short C or S-shaped [[fascicles]] or cartwheel formations
* Feathery appearance cells, with holes or tears in the tissue
* Feathery [[appearance]] [[Cells (biology)|cells]], with holes or [[tears]] in the [[Tissue (biology)|tissue]]
* Visible chromosomes in cells during cell division
* Visible [[chromosomes]] in [[Cells (biology)|cells]] during [[cell division]]
* Fine, pale, and even-looking chromatin
* Fine, pale, and even-looking [[chromatin]]
* Inflammation, accelerated mitotic index with normal mitoses
* [[Inflammation]], [[accelerated]] [[mitotic index]] with normal [[mitoses]]
* Non-pleomorphic cells with high rate of mitosis
* Non-[[pleomorphic]] [[Cells (biology)|cells]] with high rate of [[mitosis]]
* Essentially spindle cell proliferation
* Essentially [[spindle cell]] [[proliferation]]
* Stroma is rich in collagen and/or myxoid ground substance
* [[Stroma]] is rich in [[collagen]] and/or myxoid [[ground substance]]
|Positive for:
|Positive for:
* Smooth muscle actin
* [[Smooth muscle]] [[actin]]
* Muscle specific actin
* [[Muscle]] specific [[actin]]
* Vimentin
* [[Vimentin]]
* Calponin
* [[Calponin]]
* CD68  
* [[CD68]]
Negative for:
Negative for:
* S100
* [[S100A1|S100]]
* Desmin
* [[Desmin]]
* Keratin
* [[Keratin]]
* CD34
* [[CD34]]
* Caldesmon
* [[Caldesmon]]
* ALK
* ALK
* p53
* [[p53]]
|
|
* Benign
* [[Benign]]
|
|
* Reparative process secondary to trauma
* Reparative [[process]] secondary to [[trauma]]
|
|
* Anywhere in body  
* Anywhere in [[Human body|body]]
* Arms (34%)  
* Arms (34%)  
* Flexor forearm (27%)  
* [[Flexor]] [[forearm]] (27%)  
* Head and neck region (24%)  
* [[Head]] and [[neck]] region (24%)  
* Trunk (chest, back) (21%)
* [[Trunk]] ([[chest]], [[back]]) (21%)
* Thigh (17%)
* [[Thigh]] (17%)
* Legs (14%)
* [[Legs]] (14%)
* Upper arm (12%)
* [[Upper arm]] (12%)
* Hands (rarely)
* [[Hands]] (rarely)
* Bladder
* [[Urinary bladder|Bladder]]
* Breast
* [[Breast]]
* Cervix
* [[Cervix]]
* Intra-articular  
* Intra-[[articular]]
* Prostate
* [[Prostate]]
* Vagina
* [[Vagina]]
* Vulva
* [[Vulva]]
|
|
* Solitary firm non-painful mass
* [[Solitary]] firm non-[[painful]] [[mass]]
* Oval or round in shape with irregular margins
* Oval or round in [[Shape theory|shape]] with [[Irregular lesion|irregular]] margins
* Tender (maybe)
* [[Tenderness (medicine)|Tender]] (maybe)
|Also known as:
|Also known as:
* Nodular pseudosarcomatous fasciits
* [[Nodular]] pseudosarcomatous [[fasciitis]]
* Subcutaneous pseudosarcomatous fibromatosis
* [[Subcutaneous tissue|Subcutaneous]] pseudosarcomatous [[fibromatosis]]
* Pseudosarcomatous fasciitis
* Pseudosarcomatous [[fasciitis]]
|-
|-
|[[Hypertrophic scars|'''Hypertrophic scars''']]
| style="background:#DCDCDC;" align="center" + |[[Hypertrophic scars|'''Hypertrophic scars''']]
|
|
* Overexpression of growth factors such as:
* [[Overexpression]] of [[growth factors]] such as:
 
** [[TGF-beta]]
* TGF-beta
** [[VEGF]]
* VEGF
** [[CTGF]]
* CTGF
* Decreased production of [[matrix metalloproteinases]]
 
* Increased numbers of [[Platelet-derived growth factor receptor|PDGF receptors]] on [[fibroblasts]]
* Decreased production of matrix metalloproteinases
* Over-activation of [[Signals (biology)|signals]] for [[insulin-like growth factor-1]]
* Increased numbers of PDGF receptors on fibroblasts
* Decreased [[fibroblast]] [[apoptosis]] rate
* Over-activation of signals for insulin-like growth factor-1
* Increased [[expression]] of [[thymic]] [[stromal]] lymphopoietin
* Decreased fibroblast apoptosis rate
* Increased [[fibrocyte]] [[Activation energy|activation]] via [[stromal cell-derived factor-1]]
* Increased expression of thymic stromal lymphopoietin
* [[Genes]] in four susceptibility [[loci]] on [[chromosomes]] 1q41, 3q22.3-23, and 15q21.3
* Increased fibrocyte activation via stromal cell-derived factor-1  
* Genes in four susceptibility loci on chromosomes 1q41, 3q22.3-23, and 15q21.3
|
|
* Excess collagen formation with varying numbers of fibroblasts and myofibroblasts
* Excess [[collagen]] [[Formation matrix|formation]] with varying numbers of [[fibroblasts]] and [[myofibroblasts]]
* Collagen fibers are arranged in a wavy pattern predominantly oriented parallel to the epithelial surface
* [[Collagen]] [[Fiber|fibers]] are arranged in a wavy pattern predominantly oriented [[Parallel processing|parallel]] to the [[epithelial]] [[Surface area|surface]]
* Nodular structures containing myofibroblasts and vertically aligned vessels
* [[Nodular]] structures containing [[myofibroblasts]] and vertically aligned [[vessels]]
|_
|_
|
|
* Benign
* [[Benign]]
|
|
* Frequent complication of burn injuries
* Frequent [[Complication (medicine)|complication]] of [[Burn (injury)|burn injuries]]
* Piercings
* Piercings
* Cuts
* Cuts
* Surgical wounds
* [[Surgical wounds]]
* Lacerations
* [[Lacerations]]
Adverse wound healing factors at the injury site such as:
[[Adverse effect|Adverse]] [[wound healing]] factors at the [[injury]] site such as:
* Infection (acne, folliculitis, chicken pox, and vaccinations)
* [[Infection]] ([[acne]], [[folliculitis]], [[chicken pox]], and [[vaccinations]])
* Excessive tension
* Excessive [[tension]]
|
|
* Milder than keloids
* Milder than [[keloids]]
* Don’t grow beyond the boundaries of the original skin injury
* Don’t [[Growth|grow]] beyond the boundaries of the original [[skin]] [[injury]]
|
|
* Cosmetic disfigurement
* [[Cosmetics|Cosmetic]] [[disfigurement]]
* Pruritis
* [[Pruritis]]
* Pain
* [[Pain]]
* Functional impairment (from contracture or mechanical irritation)
* Functional [[impairment]] (from [[contracture]] or mechanical [[irritation]])
* Loss of temperature regulation
* Loss of [[temperature regulation]]
* Dry skin
* [[Dry skin]]
* Neuropathic pain
* [[Neuropathic pain]]
|Hypertrophic scars can be classified as:
|[[Hypertrophic scars]] can be classified as:
* Mature scar
* [[Mature]] [[scar]]
* Immature scar
* Immature [[scar]]
* Linear, hypertrophic scar (surgical/traumatic)
* Linear, [[hypertrophic scar]] ([[surgical]]/[[Trauma|traumatic]])
* Widespread, hypertrophied scar (burn)
* Widespread, [[Hypertrophic|hypertrophied]] [[scar]] ([[burn]])
|-
|-
|[[Keloids|'''Keloids''']]<ref>{{cite journal|doi=10.4197/Med. 17-3.1}}</ref><ref name="pmid20555348">{{cite journal| author=Russell SB, Russell JD, Trupin KM, Gayden AE, Opalenik SR, Nanney LB et al.| title=Epigenetically altered wound healing in keloid fibroblasts. | journal=J Invest Dermatol | year= 2010 | volume= 130 | issue= 10 | pages= 2489-96 | pmid=20555348 | doi=10.1038/jid.2010.162 | pmc=2939920 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20555348  }} </ref><ref name="pmid17989729">{{cite journal| author=Smith JC, Boone BE, Opalenik SR, Williams SM, Russell SB| title=Gene profiling of keloid fibroblasts shows altered expression in multiple fibrosis-associated pathways. | journal=J Invest Dermatol | year= 2008 | volume= 128 | issue= 5 | pages= 1298-310 | pmid=17989729 | doi=10.1038/sj.jid.5701149 | pmc=2933038 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17989729  }} </ref><ref name="HuangMurphy2013">{{cite journal|last1=Huang|first1=Chenyu|last2=Murphy|first2=George F.|last3=Akaishi|first3=Satoshi|last4=Ogawa|first4=Rei|title=Keloids and Hypertrophic Scars|journal=Plastic and Reconstructive Surgery Global Open|volume=1|issue=4|year=2013|pages=e25|issn=2169-7574|doi=10.1097/GOX.0b013e31829c4597}}</ref>
| style="background:#DCDCDC;" align="center" + |[[Keloids|'''Keloids''']]<ref>{{cite journal|doi=10.4197/Med. 17-3.1}}</ref><ref name="pmid20555348">{{cite journal| author=Russell SB, Russell JD, Trupin KM, Gayden AE, Opalenik SR, Nanney LB et al.| title=Epigenetically altered wound healing in keloid fibroblasts. | journal=J Invest Dermatol | year= 2010 | volume= 130 | issue= 10 | pages= 2489-96 | pmid=20555348 | doi=10.1038/jid.2010.162 | pmc=2939920 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20555348  }} </ref><ref name="pmid17989729">{{cite journal| author=Smith JC, Boone BE, Opalenik SR, Williams SM, Russell SB| title=Gene profiling of keloid fibroblasts shows altered expression in multiple fibrosis-associated pathways. | journal=J Invest Dermatol | year= 2008 | volume= 128 | issue= 5 | pages= 1298-310 | pmid=17989729 | doi=10.1038/sj.jid.5701149 | pmc=2933038 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17989729  }} </ref><ref name="HuangMurphy2013">{{cite journal|last1=Huang|first1=Chenyu|last2=Murphy|first2=George F.|last3=Akaishi|first3=Satoshi|last4=Ogawa|first4=Rei|title=Keloids and Hypertrophic Scars|journal=Plastic and Reconstructive Surgery Global Open|volume=1|issue=4|year=2013|pages=e25|issn=2169-7574|doi=10.1097/GOX.0b013e31829c4597}}</ref>
|
|
* Epigenetic alteration in fibroblast DNA and acetylation of histone proteins
* [[Epigenetic]] alteration in [[fibroblast]] [[DNA]] and [[acetylation]] of [[histone]] [[proteins]]
* Overexpression of growth factors such as:
* [[Overexpression]] of [[growth factors]] such as:
** TGF-beta
** [[TGF-beta]]
** VEGF
** [[VEGF]]
** CTGF
** [[CTGF]]
* Decreased production of matrix metalloproteinases
* Decreased production of [[matrix metalloproteinases]]
* Increased numbers of PDGF receptors on fibroblasts
* Increased numbers of [[Platelet-derived growth factor receptor|PDGF receptors]] on [[fibroblasts]]
* Over-activation of signals for insulin-like growth factor-1
* Over-activation of [[Signals (biology)|signals]] for [[insulin-like growth factor-1]]
* Decreased fibroblast apoptosis rate
* Decreased [[fibroblast]] [[apoptosis]] [[rate]]
* Increased expression of thymic stromal lymphopoietin
* Increased [[expression]] of [[thymic]] [[stromal]] lymphopoietin
* Increased fibrocyte activation via stromal cell-derived factor-1  
* Increased [[fibrocyte]] activation via [[stromal cell-derived factor-1]]
* Genes in following susceptibility loci on chromosomes:
* [[Genes]] in following susceptibility [[loci]] on [[chromosomes]]:
** 1q41
** 1q41
** 3q22.3-23
** 3q22.3-23
Line 1,477: Line 1,510:
** 7p11
** 7p11
** 10q23.31
** 10q23.31
* In Japanese population, 4 SNP loci (rs873549, rs1511412, rs940187, and rs8032158) in 3 chromosomal regions (1q41, 3q22.3–23, and 15q21.3) exhibit significant associations with keloids
* In Japanese [[population]], 4 SNP loci (rs873549, rs1511412, rs940187, and rs8032158) in 3 [[chromosomal]] regions (1q41, 3q22.3–23, and 15q21.3) exhibit [[Significant figure|significant]] [[Association (statistics)|associations]] with [[keloids]]
|
|
*Hyalinized collagen bundles
*Hyalinized [[collagen]] bundles
* Excess collagen formation with varying numbers of fibroblasts and myofibroblasts
* Excess [[collagen]] [[Formation matrix|formation]] with varying numbers of [[fibroblasts]] and [[myofibroblasts]]
* Hypocellular zones of fibrous tissue containing thickened, glassy, eosinophilic collagen bundles
* Hypocellular zones of [[fibrous tissue]] containing thickened, glassy, [[eosinophilic]] [[collagen]] bundles
|_
|_
|
|
* Benign
* [[Benign]]
|
|
* Minor injuries
* [[Minor Injuries Unit|Minor injuries]]
* Insect bites
* [[Insect bites]]
* Pimples
* [[Pimples]]
* Earlobe piercings
* [[Earlobe]] piercings
Adverse wound healing factors at the injury site such as:
Adverse [[wound healing]] factors at the [[injury]] site such as:
* Infection
* [[Infection]]
* Inflammation
* [[Inflammation]]
* Excessive tension
* Excessive [[tension]]
* Excessive motion (such as in injuries over a joint),
* Excessive [[Motion (physics)|motion]] (such as in [[injuries]] over a [[joint]]),
* Wound left to heal without stitches
* [[Wound]] left to [[Healing|heal]] without [[Stitch|stitches]]
Keloids may be associated with rare inherited syndromes such as:
[[Keloids]] may be associated with [[rare]] [[inherited]] [[syndromes]] such as:
* Rubinstein-Taybi syndrome
* [[Rubinstein-Taybi syndrome]]
* Goeminne syndrome
* Goeminne [[syndrome]]
|
|
* Any part of body having injury
* Any part of [[Human body|body]] having [[injury]]
* Upper chest
* Upper [[chest]]
* Shoulders
* [[Shoulders]]
* Upper back  
* Upper [[back]]
* Head and neck (especially on ears)
* [[Head]] and [[neck]] (especially on [[ears]])
|
|
* Cosmetic disfigurement
* [[Cosmetics|Cosmetic]] [[disfigurement]]
* Raised, reddish nodules
* Raised, reddish [[nodules]]
* Skin pigmentation
* [[Skin pigmentation]]
* Itchiness
* [[Itchiness]]
* Redness
* [[Redness of the skin|Redness]]
* Unusual sensations
* Unusual [[sensations]]
* Pain
* [[Pain]]
* Functional impairment
* Functional [[impairment]]
|
|
* Keloid is Greek for "crab claw"
* [[Keloid]] is [[Greek key|Greek]] for "crab claw"
 
* [[Extend]] beyond the borders of the original [[wound]]
* Extend beyond the borders of the original wound
* [[Regression|Regress]] spontaneously
* Regress spontaneously
* [[Recurrence plot|Recur]] after [[excision]]
* Recur after excision
* Darkly [[Pigmented Lesions|pigmented]] people/African-American, Asians (Han Chinese, Japanese) are more [[prone]] to form [[keloids]]
* Darkly pigmented people/African-American, Asians (Han Chinese, Japanese) are more prone to form keloids
[[Keloids]] can be [[Prevention|prevented]] by using:  
Keloids can be prevented by using:  
* [[Pressure]] [[Dressing (medical)|dressing]]
* Pressure dressing
* [[Silicones|Silicone]] [[gel]] pad
* Silicone gel pad
* Paper tape over the [[injury]] site for 23-24hours per day
* Paper tape over the injury site for 23-24hours per day
[[Keloids]] can be classified as:
Keloids can be classified as:
* Minor [[keloid]]
* Minor keloid
* Major [[keloid]]
* Major keloid
|-
|-
|[[Primitive neuroectodermal tumor|'''Primitive neuroectodermal tumor''']] (PNET)<ref name="pmid21837678">{{cite journal| author=Smoll NR| title=Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs). | journal=Cancer | year= 2012 | volume= 118 | issue= 5 | pages= 1313-22 | pmid=21837678 | doi=10.1002/cncr.26387 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21837678  }} </ref><ref name="pmid8129041">{{cite journal| author=Eibl RH, Kleihues P, Jat PS, Wiestler OD| title=A model for primitive neuroectodermal tumors in transgenic neural transplants harboring the SV40 large T antigen. | journal=Am J Pathol | year= 1994 | volume= 144 | issue= 3 | pages= 556-64 | pmid=8129041 | doi= | pmc=1887088 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8129041  }} </ref><ref name="pmid1933879">{{cite journal| author=Ohgaki H, Eibl RH, Wiestler OD, Yasargil MG, Newcomb EW, Kleihues P| title=p53 mutations in nonastrocytic human brain tumors. | journal=Cancer Res | year= 1991 | volume= 51 | issue= 22 | pages= 6202-5 | pmid=1933879 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1933879  }} </ref>
| style="background:#DCDCDC;" align="center" + |[[Primitive neuroectodermal tumor|'''Primitive neuroectodermal tumor''']] ([[PNET]])<ref name="pmid21837678">{{cite journal| author=Smoll NR| title=Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs). | journal=Cancer | year= 2012 | volume= 118 | issue= 5 | pages= 1313-22 | pmid=21837678 | doi=10.1002/cncr.26387 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21837678  }} </ref><ref name="pmid8129041">{{cite journal| author=Eibl RH, Kleihues P, Jat PS, Wiestler OD| title=A model for primitive neuroectodermal tumors in transgenic neural transplants harboring the SV40 large T antigen. | journal=Am J Pathol | year= 1994 | volume= 144 | issue= 3 | pages= 556-64 | pmid=8129041 | doi= | pmc=1887088 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8129041  }} </ref><ref name="pmid1933879">{{cite journal| author=Ohgaki H, Eibl RH, Wiestler OD, Yasargil MG, Newcomb EW, Kleihues P| title=p53 mutations in nonastrocytic human brain tumors. | journal=Cancer Res | year= 1991 | volume= 51 | issue= 22 | pages= 6202-5 | pmid=1933879 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1933879  }} </ref>
|
|
*EWS / FLI1 fusion product by RT-PCR due to t(11;22)(q24/q22;q12) (90%)
*[[Ewing sarcoma breakpoint region 1|EWS]] / [[FLI1]] [[Fusion gene|fusion]] [[Product (biology)|product]] by [[Reverse transcription polymerase chain reaction|RT-PCR]] due to t(11;22)(q24/q22;q12) (90%)
*p53 tumor suppressor gene mutations
*[[p53]] [[tumor suppressor gene]] [[mutations]]
|
|
* Small blue cell tumor
* Small blue [[Cell (biology)|cell]] [[tumor]]
* Round hyperchromatic cells
* Round hyperchromatic [[Cells (biology)|cells]]
* Abundant mitotic figures
* Abundant [[mitotic]] figures
* Homer Wright rosettes
* Homer Wright rosettes
* Fibrosis
* [[Fibrosis]]
* Neurosecretory granules on electron microscopy
* Neurosecretory [[granules]] on [[electron microscopy]]
|Positive for:
|Positive for:
* CD99  
* [[CD99]]
* FLI1
* [[FLI1]]
* PAS+ diastase sensitive (glycogen)
* [[PAS stain|PAS]]+ [[diastase]] sensitive ([[glycogen]])
* Vimentin
* [[Vimentin]]
* Neuron specific enolase
* [[Neuron]] specific [[enolase]]
Negative for:
Negative for:
* Cytokeratin (usually)
* [[Cytokeratin]] (usually)
* Desmin
* [[Desmin]]
* WT1
* [[WT1]]
* GFAP
* [[Glial fibrillary acidic protein|GFAP]]
* PAX2
* [[PAX2]]
|
|
* Malignant [[neural crest]] tumor
* [[Malignant]] [[neural crest]] [[tumor]]
* Highly aggressive (rapid death within 1 year)
* Highly aggressive (rapid death within 1 year)
* Metastases to lung, pleura, bone and liver
* [[Metastases]] to [[lung]], [[pleura]], [[bone]] and [[liver]]
|
|
* [[Gorlin syndrome]]
* [[Gorlin syndrome]]
Line 1,565: Line 1,597:
* [[Rubinstein-Taybi syndrome]]
* [[Rubinstein-Taybi syndrome]]
|
|
* CNS PNET: Intracranial (cerebral hemisphere)
* [[CNS]] [[PNET]]: Intracranial ([[cerebral hemisphere]])
* Peripheral PNET: kidneys
* Peripheral [[PNET]]: [[kidneys]]
|
|
* Asymptomatic for years
* [[Asymptomatic]] for years
* [[Morning headache]]
* [[Morning headache]]
* [[Restlessness]]
* [[Restlessness]]
Line 1,575: Line 1,607:
* Frequent falls
* Frequent falls
* Positional [[dizziness]]
* Positional [[dizziness]]
* Increased intracranial pressure
* Increased [[intracranial pressure]]
* Cranial nerve palsy
* [[Seizures]]
* Seizures
* [[Papilledema]]
* [[Papilledema]]
* [[Strabismus]]
* [[Strabismus]]
* [[Nystagmus]]
* [[Nystagmus]]
* [[Ataxia]]
* [[Ataxia]]
* Motor [[weakness]]
* [[Motor skill|Motor]] [[weakness]]
* Facial [[sensory loss]]
* [[Facial]] [[sensory loss]]
* Third, fourth, and sixth [[cranial nerve palsies]]
* [[Third cranial nerve palsy|Third]], [[Fourth (trochlear) nerve palsy|fourth]], and [[Sixth (abducent) nerve palsy|sixth]] [[cranial nerve palsies]]
* Abdominal/flank pain
* [[Abdominal]]/[[flank pain]]
* Hematuria
* [[Hematuria]]
|Also known as:
|Also known as:
* CNS PNET
* [[CNS]] [[PNET]]
* Askin tumor
* Askin [[tumor]]
* Peripheral neuroepithelioma
* Peripheral [[Neuroepitheliomatous|neuroepithelioma]]
* Ependymoblastoma
* [[Ependymoblastoma]]
WHO classified PNET into 3 subtypes:
[[World Health Organization|WHO]] classified [[PNET]] into 3 subtypes:
* Central primitive neuroectodermal tumors (PNETs)
* [[Central]] [[primitive neuroectodermal tumors]] ([[PNET|PNETs]])
* Neuroblastomas
* [[Neuroblastomas]]
* Peripheral primitive neuroectodermal tumors (pPNETs)
* Peripheral [[primitive neuroectodermal tumors]] (p[[PNET]]<nowiki/>s)
Peripheral PNET (pPNET) is now thought to be virtually identical to Ewing sarcoma
Peripheral [[PNET]] (p[[PNET]]) is now thought to be virtually identical to [[Ewing sarcoma]]
|}
|}



Latest revision as of 01:43, 29 April 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]

Overview

Extra-abdominal desmoid tumor must be differentiated from fibrosarcoma, low-grade fibromyxoid sarcoma and Gardner fibroma. Intra-abdominal desmoid tumor must be differentiated from gastrointestinal stromal tumor (GIST), benign fibrous tumor/solitary fibrous tumor (SFT), inflammatory myofibroblastic tumor (IMT), sclerosing mesenteritis and retroperitoneal fibrosis. Furthermore, generally all desmoid tumors must be differentiated from acute hematoma, lymphoma, rhabdomyosarcoma, liposarcoma, leiomyosarcoma, neurofibroma, nodular fasciitis, hypertrophic scars,keloids and primitive neuroectodermal tumor.

Differentiating Desmoid tumor from other Diseases

Differentiating desmoid tumor from other diseases
Disease entity Etiology (Genetic or others) Histopathological findings Immunohistochemical staining Benign/Malignant Risk factors Common site of involvement Clinical manifestations Other associated features
Desmoid tumor Sporadic desmoids are associated with following mutations:

Familial desmoids/Hereditary desmoid disease is associated with:

Pediatric desmoids have following additional mutations involving:

Histologically, desmoid tumors consist of: Positive for:

Negative for:

Desmoids may be associated with following:
Fibrosarcoma/Fibroblastic sarcoma Strongly positive for:

Negative for:

Primary bone malignancy involving end of long bones:
Low-grade fibromyxoid sarcoma[4][5][9][10][11][12][13][14] Translocation:
  • t(7;18;16) or
  • t(7;16) (q34:p11)

Fusion gene:

Positive for:
  • MUC4 (highly specific and sensitive)
  • Vimentin

Occasionally positive for:

Negative for:

_
Majority occurring in subfascial location and rarely involving subcutis or dermis in following sites:
  • Painless, slow-growing well-circumscribed mass
  • Average size-5cm (ranges from 1-20cm)
_
Benign fibrous tumor/Solitary fibrous tumor (SFT) [15][16][17][18][19][20][21][22][23] Intra-chromosomal inversion at:

TERT promoter mutations responsible for:

Positive for:

In few cases, positive for:

Negative for:

_ Paraneoplastic syndromes associated with SFTs include:
Gardner fibroma/Gardner-associated fibroma (GAF)[24][25][6][26] Positive for: Any part of body:
Gastrointestinal stromal tumor (GIST)[27][28][29][30][31] Activating mutation of:

Pediatric GISTs (SDH-deficient) have mutations of one of the:

GIST as a part of Carney triad has following mutation:

Wild-type GISTs have following three molecular subtypes:

GIST cells basically arise from interstitial cells of Cajal (ICCs) and appear as follows on histology: Positive for:

Consistently negative for:

Can involve any part of GIT: Depending on tumor size and localization in GIT: _
Inflammatory myofibroblastic tumor (IMT)[32][33][34][35][36][37][38][39][33][36][7] Unknown underlying etiology, may be due to inflammatory reaction to:

Mutations such as:

Positive for:

Negative for:

Also known as:
Sclerosing mesenteritis[40][41][42][43][44][45][46][47][48][49][50][51][52]

[53][54][55][56][57][58][59][60]

Unknown exact etiology: Varying components of: _ Nomenclature used in the medical literature for idiopathic primary inflammatory and fibrotic processes of the mesentery is as follows:
Retroperitoneal fibrosis[61][62][63][64][65][66][67][68][69][70][71][72][73]

Idiopathic (70%) [Ormond's disease] is an immune-mediated disease caused by:

Secondary to:

Fibro-inflammatory disease histologically hallmarked by fibrous tissue and chronic inflammation: Retroperitoneal fibrosis is also known as:
Lymphoma Diffuse large B cell lymphoma (DLBCL):

Extranodal marginal B cell lymphoma:

Follicular lymphoma:

Burkitt lymphoma:

Positive for:

Negative for:

Anywhere in body WHO classification of lymphoma:
Rhabdomyosarcoma (RMS)[74][75][76][77][78][79][80][81][82][83] Histologic classification: Positive for:

Maybe positive for:

Histology of RMS similar to that of other childhood small round blue cell tumors involving bone and soft tissue such as:
Liposarcoma[84][85][86][87][88][89][90][91][92][93] Atypical lipomatous tumor/well differentiated liposarcoma and dedifferentiated liposarcoma are associated with:

Myxoid liposarcoma is associated with:

Pleomorphic liposarcoma is associated with:

Well-differentiated liposarcoma:

De-differentiated liposarcoma:

Atypical lipomatous tumor/well differentiated liposarcoma is positive for: _
Leiomyosarcoma[94][95][96][97][98][99][100][101][102] Positive for:

Negative for:

_
Neurofibroma[103][104][105][106][107][108][109][106][110][111][112] Can be sporadic or as a part of Neurofibromatosis 1 and 2 Positive for:

Negative for:

Nodular fasciitis[113][114][115][116][117][118][119][120][121][122] Positive for:

Negative for:

Also known as:
Hypertrophic scars _

Adverse wound healing factors at the injury site such as:

Hypertrophic scars can be classified as:
Keloids[123][124][125][126] _

Adverse wound healing factors at the injury site such as:

Keloids may be associated with rare inherited syndromes such as:

Keloids can be prevented by using:

Keloids can be classified as:

Primitive neuroectodermal tumor (PNET)[127][128][129] Positive for:

Negative for:

Also known as:

WHO classified PNET into 3 subtypes:

Peripheral PNET (pPNET) is now thought to be virtually identical to Ewing sarcoma

Reference

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