Pseudomyxoma peritonei pathophysiology: Difference between revisions

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{{Pseudomyxoma peritonei}}
{{Pseudomyxoma peritonei}}
{{CMG}}{{AE}}{{Nnasiri}}{{PSD}}
{{CMG}}{{AE}}{{Nnasiri}}
==Overview==
==Overview==
Pseudomyxoma peritonei (PMP) is a rare tumor known for its production of abundant mucinous ascites in the abdominal cavity. It can have a mass impact on vital organs such as spleen, pancreas, kidney. Pseudomyxoma peritonei may be divided into two pathological subtypes: Peritoneal adenomucinosis and Peritoneal mucinous carcinoma.
Pseudomyxoma peritonei (PMP) is a rare [[tumor]] known for its production of abundant [[mucinous]] [[ascites]] in the [[abdominal cavity]]. It can have a mass impact on vital organs such as [[spleen]], [[pancreas]], and [[kidney]]. Pseudomyxoma peritonei may be divided into two [[pathological]] subtypes: [[peritoneal]] adenomucinosis and [[Peritoneal mucinous carcinomatosis|peritoneal mucinous carcinoma.]]


==Pathogenesis==
==Pathogenesis==
The pathogenesis of the disease is related to [[biomarkers]] and molecular [[genetic]] alterations.  
The pathogenesis of the disease is related to [[biomarkers]] and molecular [[genetic]] alterations.  
*Immunohistochemica<nowiki/>l markers and [[genetic]] alterations involved in the [[pathogenesis]] of pseudomyxoma peritonei include:<ref name="pmid10362811">{{cite journal |vauthors=Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM |title=Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women |journal=Am. J. Pathol. |volume=154 |issue=6 |pages=1849–55 |date=June 1999 |pmid=10362811 |pmc=1866622 |doi=10.1016/S0002-9440(10)65442-9 |url=}}</ref>
*[[Immunohistochemistry|Immunohistochemica]]<nowiki/>[[Immunohistochemistry|l markers]] and [[genetic]] alterations involved in the [[pathogenesis]] of pseudomyxoma peritonei include:<ref name="pmid10362811">{{cite journal |vauthors=Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM |title=Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women |journal=Am. J. Pathol. |volume=154 |issue=6 |pages=1849–55 |date=June 1999 |pmid=10362811 |pmc=1866622 |doi=10.1016/S0002-9440(10)65442-9 |url=}}</ref>
**[[CK 20]]
**CK 20  
**[[CDX2]] and [[MUC2]] are found to be positive in these tumors.  
**[[CDX2]] and [[MUC2]] are found to be positive in these [[Tumor|tumors]].  
**[[KRAS]] [[Mutations|mutation]] and [[loss of heterozygosity]] in some gene loci.
**[[KRAS]] [[Mutations|mutation]] and [[loss of heterozygosity]] in some [[Loci|gene loci]].
**Losses of [[alleles]] in [[chromosomes]] [[18q syndrome|18q]], 17p, 5q.
**Losses of [[alleles]] in [[chromosomes]] [[18q syndrome|18q]], 17p, 5q.


==Pathology==
==Pathology==
Pseudomyxoma peritonei may be divided into two pathological subtypes:<ref name="pmid7503361">{{cite journal |vauthors=Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM |title=Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to "pseudomyxoma peritonei" |journal=Am. J. Surg. Pathol. |volume=19 |issue=12 |pages=1390–408 |date=December 1995 |pmid=7503361 |doi= |url=}}</ref><ref name="pmid22718846">{{cite journal |vauthors=Carr NJ, Finch J, Ilesley IC, Chandrakumaran K, Mohamed F, Mirnezami A, Cecil T, Moran B |title=Pathology and prognosis in pseudomyxoma peritonei: a review of 274 cases |journal=J. Clin. Pathol. |volume=65 |issue=10 |pages=919–23 |date=October 2012 |pmid=22718846 |doi=10.1136/jclinpath-2012-200843 |url=}}</ref>
Pseudomyxoma peritonei may be divided into two pathological subtypes:<ref name="pmid7503361">{{cite journal |vauthors=Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM |title=Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to "pseudomyxoma peritonei" |journal=Am. J. Surg. Pathol. |volume=19 |issue=12 |pages=1390–408 |date=December 1995 |pmid=7503361 |doi= |url=}}</ref>
*[[Disseminated peritoneal adenomucinosis]] (DPAM) which has charasteric features of followings:
*[[Disseminated peritoneal adenomucinosis]] (DPAM) which has charracteristic features of the following:
**[[peritoneal]] lesions with abundant extracellular [[mucin]]
**[[Peritoneal]] lesions with abundant [[extracellular]] [[mucin]]
**Proliferative mucinous epithelium  
**Proliferative [[mucinous]] [[epithelium]]
**Less [[mitotic]] activity compare with peritoneal mucinous carcinomatosis.
**Less [[mitotic]] activity as compared with [[Peritoneum|peritoneal]] [[mucinous]] [[carcinomatosis]]
*[[Peritoneal mucinous carcinomatosis]] (PMCA) which is characterized by:
*[[Peritoneal mucinous carcinomatosis]] (PMCA) which is characterized by:
**[[Peritoneal]] lesions having more abundant mucinous [[epithelium]]
**[[Peritoneal]] lesions having more abundant [[mucinous]] [[epithelium]]
**Characteristic cytologic features of [[carcinoma]], with high mitotic activity.
**Characteristic [[Cytological|cytologic]] features of [[carcinoma]], with high [[Mitotic|mitotic activity]].


==Immunohistology==
==Immunohistology==
Immunohistochemical markers can help identify the organ of origin.  
[[Immunohistochemistry|Immunohistochemical markers]] can help identify the organ of origin.  
*The tumor is positive for cytokeratin 20 (CK20), [[CEA]], caudal-type homeobox protein 2 (CDX-2), and [[MUC2]] as well as negative cytokeratin 7 (CK7) and [[CA125]].  
*The tumor is positive for [[cytokeratin]] 20 (CK20), [[CEA]], caudal-type homeobox protein 2 (CDX-2), and [[MUC2]] as well as negative [[cytokeratin]] 7 (CK7) and [[CA125]].  
*Studies have shown that mucin [[MUC2]] and MUC5AC is extensively positive in pseudomyxoma peritonei patients. <ref name="pmid16978201">{{cite journal |vauthors=Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M |title=CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases |journal=Histopathology |volume=49 |issue=4 |pages=381–7 |date=October 2006 |pmid=16978201 |doi=10.1111/j.1365-2559.2006.02512.x |url=}}</ref>
*Studies have shown that mucin [[MUC2]] and MUC5AC is extensively positive in pseudomyxoma peritonei patients. <ref name="pmid16978201">{{cite journal |vauthors=Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M |title=CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases |journal=Histopathology |volume=49 |issue=4 |pages=381–7 |date=October 2006 |pmid=16978201 |doi=10.1111/j.1365-2559.2006.02512.x |url=}}</ref>


== References ==
== References ==
{{Reflist|1}}
{{Reflist|2}}


[[Category:Types of cancer]]
[[Category:Types of cancer]]

Latest revision as of 20:30, 1 April 2019

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

Overview

Pseudomyxoma peritonei (PMP) is a rare tumor known for its production of abundant mucinous ascites in the abdominal cavity. It can have a mass impact on vital organs such as spleen, pancreas, and kidney. Pseudomyxoma peritonei may be divided into two pathological subtypes: peritoneal adenomucinosis and peritoneal mucinous carcinoma.

Pathogenesis

The pathogenesis of the disease is related to biomarkers and molecular genetic alterations.

Pathology

Pseudomyxoma peritonei may be divided into two pathological subtypes:[2]

Immunohistology

Immunohistochemical markers can help identify the organ of origin.

  • The tumor is positive for cytokeratin 20 (CK20), CEA, caudal-type homeobox protein 2 (CDX-2), and MUC2 as well as negative cytokeratin 7 (CK7) and CA125.
  • Studies have shown that mucin MUC2 and MUC5AC is extensively positive in pseudomyxoma peritonei patients. [3]

References

  1. Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM (June 1999). "Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women". Am. J. Pathol. 154 (6): 1849–55. doi:10.1016/S0002-9440(10)65442-9. PMC 1866622. PMID 10362811.
  2. Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM (December 1995). "Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to "pseudomyxoma peritonei"". Am. J. Surg. Pathol. 19 (12): 1390–408. PMID 7503361.
  3. Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M (October 2006). "CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases". Histopathology. 49 (4): 381–7. doi:10.1111/j.1365-2559.2006.02512.x. PMID 16978201.


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