Vascular tumor: Difference between revisions

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==Overview==
==Overview==


'''Vascular tumor''' may mean:
'''[[Vascular]] [[tumors]]''' are growths ([[tumor#Tumor Types: Malignant vs. benign|benign or malignant]]) formed from [[blood vessel]]s; for example, [[hemangioma]], [[hemangioendothelioma]], [[Kaposi sarcoma]], [[angiosarcoma]]. '''International Society for the Study of Vascular Anomalies (ISSVA)''' has classified [[vascular tumors]] into three main categories depending on their clinical and histological behavior. Some [[lesions]] related clinically and/or histologically to [[vascular]] [[tumors]] have been described in '''[[provisionally unclassified vascular anomalies]]''' and '''[[related lesions]]'''.
 
* '''[[tumor]] of vascular origin''', a growth ([[tumor#Tumor Types: Malignant vs. benign|benign or malignant]]) formed from [[blood vessel]]s; for example, [[hemangioma]], [[hemangioendothelioma]], [[Kaposi sarcoma]], [[angiosarcoma]], etc.
* '''International Society for the Study of Vascular Anomalies (ISSVA)''' has classified [[vascular tumors]] into three categories depending on their clinical and histological behavior. Some [[lesions]] related clinically and/or histologically to [[vascular]] tumors have been described as '''related lesions'''.


==Classification==
==Classification==
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=Benign vascular tumors=
=Benign vascular tumors=
For details about benign vascular tumors, [[Benign vascular tumor#Benign vascular tumor|Click here]].
''' [[Benign]] [[vascular]] [[tumors]]''', are [[benign]] growths formed from [[blood vessel]]s; such as, [[hemangioma]], [[hemangioendothelioma]], [[Kaposi sarcoma]]. They exhibit a wide range of clinical manifestations, and may occur as isolated [[lesions]] or may occur as manifestation of multi-system [[syndromes]] and [[diseases]]. Their [[diagnosis]] and management depends on their clinical manifestations and coexistent [[anomalies]]. '''International Society for the Study of Vascular Anomalies (ISSVA)''' has classified these [[lesions]] into [[benign vascular tumors]] and related [[lesions]].
 
For more information on benign vascular tumors, [[Benign vascular tumor#Benign vascular tumor|click here]].


=Locally aggressive or borderline vascular tumors=
=Locally aggressive or borderline vascular tumors=
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* [[Somatic]] activating GNA14 c.614A>T (p.Gln205Leu) [[mutations]] have been found in some KHE.<ref name="pmid27476652">{{cite journal |vauthors=Lim YH, Bacchiocchi A, Qiu J, Straub R, Bruckner A, Bercovitch L, Narayan D, McNiff J, Ko C, Robinson-Bostom L, Antaya R, Halaban R, Choate KA |title=GNA14 Somatic Mutation Causes Congenital and Sporadic Vascular Tumors by MAPK Activation |journal=Am. J. Hum. Genet. |volume=99 |issue=2 |pages=443–50 |date=August 2016 |pmid=27476652 |pmc=4974082 |doi=10.1016/j.ajhg.2016.06.010 |url=}}</ref>
* [[Somatic]] activating GNA14 c.614A>T (p.Gln205Leu) [[mutations]] have been found in some KHE.<ref name="pmid27476652">{{cite journal |vauthors=Lim YH, Bacchiocchi A, Qiu J, Straub R, Bruckner A, Bercovitch L, Narayan D, McNiff J, Ko C, Robinson-Bostom L, Antaya R, Halaban R, Choate KA |title=GNA14 Somatic Mutation Causes Congenital and Sporadic Vascular Tumors by MAPK Activation |journal=Am. J. Hum. Genet. |volume=99 |issue=2 |pages=443–50 |date=August 2016 |pmid=27476652 |pmc=4974082 |doi=10.1016/j.ajhg.2016.06.010 |url=}}</ref>
* Invasion of [[bone]], [[retroperitoneum]], and [[mediastinum]] has occured in some cases but no case of [[metastasis]] has been reported yet.<ref name="pmid59885">{{cite journal |vauthors= |title=Letter: Prevention of coronary heart-disease |journal=Lancet |volume=2 |issue=7980 |pages=313–4 |date=August 1976 |pmid=59885 |doi= |url=}}</ref>
* Invasion of [[bone]], [[retroperitoneum]], and [[mediastinum]] has occured in some cases but no case of [[metastasis]] has been reported yet.<ref name="pmid59885">{{cite journal |vauthors= |title=Letter: Prevention of coronary heart-disease |journal=Lancet |volume=2 |issue=7980 |pages=313–4 |date=August 1976 |pmid=59885 |doi= |url=}}</ref>
* [[Diagnostic]] work up may include blood tests, [[biopsy]], [[contrast enhanced ultrasound]] and [[MRI]] or [[CT scan imaging]].
* [[Diagnostic]] work up may include blood tests, [[biopsy]], [[Ultrasound|contrast enhanced ultrasound]] and [[MRI]] or [[CT scan|CT scan imaging]].
* Treatment Options include [[steroid]], [[vincristine]], [[interferon alpha]], [[anti-platelet agents]], sirolimus-containing therapies and [[surgery]].<ref name="pmid30054848">{{cite journal| author=Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Häberle B| title=Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. | journal=World J Pediatr | year= 2018 | volume= 14 | issue= 4 | pages= 322-329 | pmid=30054848 | doi=10.1007/s12519-018-0171-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30054848  }} </ref>
* Treatment options include [[steroid]], [[vincristine]], [[interferon alpha]], anti-platelet agents, [[sirolimus]]-containing therapies, and [[surgery]].<ref name="pmid30054848">{{cite journal| author=Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Häberle B| title=Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. | journal=World J Pediatr | year= 2018 | volume= 14 | issue= 4 | pages= 322-329 | pmid=30054848 | doi=10.1007/s12519-018-0171-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30054848  }} </ref>
For more information on [[Kaposiform hemangioendothelioma]], [[Kaposiform hemangioendothelioma#Kaposiform hemangioendothelioma|click here]].
For more information on [[Kaposiform hemangioendothelioma]], [[Kaposiform hemangioendothelioma#Kaposiform hemangioendothelioma|click here]].


===Retiform hemangioendothelioma===
===Retiform hemangioendothelioma===
* First described in 1994 as a form of low grade [[angiosarcoma]], retiform hemangioendothelioma commonly presents as a slow growing [[asymptomatic]] solitary nodule or plaque on distal [[extremities]] in 2nd-4th decade of life.<ref name="pmid25484427">{{cite journal |vauthors=Ranga SM, Kuchangi NC, Shankar VS, Amita K, Haleuoor BB, Belagola SD |title=Retiform hemangioendothelioma: an uncommon pediatric vascular neoplasm |journal=Indian J Dermatol |volume=59 |issue=6 |pages=633 |date=November 2014 |pmid=25484427 |pmc=4248535 |doi=10.4103/0019-5154.143583 |url=}}</ref>
* First described in 1994 as a form of low grade [[angiosarcoma]], retiform hemangioendothelioma commonly presents as a slow growing [[asymptomatic]] solitary nodule or plaque on distal [[extremities]] in 2nd-4th decade of life.<ref name="pmid25484427">{{cite journal |vauthors=Ranga SM, Kuchangi NC, Shankar VS, Amita K, Haleuoor BB, Belagola SD |title=Retiform hemangioendothelioma: an uncommon pediatric vascular neoplasm |journal=Indian J Dermatol |volume=59 |issue=6 |pages=633 |date=November 2014 |pmid=25484427 |pmc=4248535 |doi=10.4103/0019-5154.143583 |url=}}</ref>
* Must be differentiated from [[angiosarcoma]].
* It must be differentiated from [[angiosarcoma]].
* High level of local [[recurrence]] but very low potential for [[metastasis]].
* High level of local [[recurrence]] but very low potential for [[metastasis]].
* [[Diagnostic]] work up includes histopathological studies, that shows branching [[blood]] [[vessels]] are arranged in [[retiform]] pattern, and MRI.
* [[Diagnostic]] work up includes histopathological studies, that shows branching [[blood]] [[vessels]] are arranged in retiform pattern and MRI.
* [[Surgery]] is the treatment of choice, though 2/3rd cases recur.
* [[Surgery]] is the treatment of choice, though 2/3rd cases recur.
* Adjuvant [[radiotherapy]] and adjuvant [[chemotherapy]] with recombinant [[interferon alpha]] and low dose [[cisplatin]] have also been reported in selected cases.  
* Adjuvant [[radiotherapy]] and adjuvant [[chemotherapy]] with recombinant [[interferon alpha]] and low dose [[cisplatin]] have also been reported in selected cases.  


===Papillary intralymphatic angioendothelioma (PILA), Dabska tumor===
===Papillary intralymphatic angioendothelioma (PILA), Dabska tumor===
* First described in 1969 by Dabska,this rare [[vascular]] [[neoplasm]] generally occurs in soft tissues but can also occur in [[bone]]. They usually appear as painless inflammatory irregular or nodular [[lesions]] below the [[skin]] surface.
* First described in 1969 by Dabska, this rare [[vascular]] [[neoplasm]] generally occurs in soft tissues but can also occur in [[bone]]. They usually appear as painless inflammatory irregular or nodular [[lesions]] below the [[skin]] surface.
* The distinctive feature on histopathology is the intravascular growth of well-differentiated [[endothelial cells]] presenting as a matchstick columnar configuration.<ref>© 1999 Lippincott Williams & Wilkins, Inc.</ref>
* The distinctive feature on histopathology is the intravascular growth of well-differentiated [[endothelial cells]] presenting as a matchstick columnar configuration.<ref>© 1999 Lippincott Williams & Wilkins, Inc.</ref>
* They are locally aggressive but rarely [[metastasize]]. Locally [[recurrence]] after [[surgery]] is very common.
* They are locally aggressive but rarely [[metastasize]]. Locally recurrence after [[surgery]] is very common.
* [[Diagnostic]] studies may include histopathological studies, [[fine needle aspiration]], [[MRI]] and [[CT scan]].<ref>https://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/</ref>
* [[Diagnostic]] studies may include histopathological studies, [[fine needle aspiration]], [[MRI]], and [[CT scan]].<ref>https://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/</ref>
* Wide local excision is the treatment of choice. However any combination of [[steroids]], [[chemotherapy]], [[radiation]] therapy, and invasive procedures can be used to treat this tumor.
* Wide local excision is the treatment of choice. However any combination of [[steroids]], [[chemotherapy]], [[radiation]] therapy, and invasive procedures can be used to treat this tumor.


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===Pseudomyogenic hemangioendothelioma===
===Pseudomyogenic hemangioendothelioma===
* A locally aggressive [[tumor]] with endothelial differentiation that usually presents as multiple [[asymptomatic]] discontinuous [[lesions]], often at [[extremities]].<ref name="pmid29511030">{{cite journal |vauthors=van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG |title=Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma |journal=Clin. Cancer Res. |volume=24 |issue=11 |pages=2678–2687 |date=June 2018 |pmid=29511030 |doi=10.1158/1078-0432.CCR-17-3512 |url=}}</ref><ref name="pmid29406432">{{cite journal |vauthors=Raftopoulos E, Royer M, Warren M, Zhao J, Rush W |title=Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature |journal=Am J Dermatopathol |volume=40 |issue=8 |pages=597–601 |date=August 2018 |pmid=29406432 |doi=10.1097/DAD.0000000000001104 |url=}}</ref>
* A locally aggressive [[tumor]] with [[endothelial]] differentiation that usually presents as multiple [[asymptomatic]] discontinuous [[lesions]], often at [[extremities]].<ref name="pmid29511030">{{cite journal |vauthors=van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG |title=Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma |journal=Clin. Cancer Res. |volume=24 |issue=11 |pages=2678–2687 |date=June 2018 |pmid=29511030 |doi=10.1158/1078-0432.CCR-17-3512 |url=}}</ref><ref name="pmid29406432">{{cite journal |vauthors=Raftopoulos E, Royer M, Warren M, Zhao J, Rush W |title=Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature |journal=Am J Dermatopathol |volume=40 |issue=8 |pages=597–601 |date=August 2018 |pmid=29406432 |doi=10.1097/DAD.0000000000001104 |url=}}</ref>
* SERPINE1-FOSB fusions are characteristic that result in over-expression of truncated form of FOSB. FBJ murine osteosarcoma viral oncogene homolog B, also known as Finkel-Biskis-Jinkins murine osteosarcoma viral oncogene homolog B, FOSB or FosB, is a [[protein]] that, in humans have been implicated as regulators of [[cell proliferation]], [[differentiation]], and transformation.<ref name="pmid29511030">{{cite journal |vauthors=van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG |title=Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma |journal=Clin. Cancer Res. |volume=24 |issue=11 |pages=2678–2687 |date=June 2018 |pmid=29511030 |doi=10.1158/1078-0432.CCR-17-3512 |url=}}</ref>
* SERPINE1-FOSB fusions are characteristic that result in over-expression of truncated form of FOSB. FBJ murine osteosarcoma viral oncogene homolog B, also known as Finkel-Biskis-Jinkins murine osteosarcoma viral oncogene homolog B, FOSB or FosB, is a [[protein]] that, in humans have been implicated as regulators of [[cell proliferation]], [[differentiation]], and transformation.<ref name="pmid29511030">{{cite journal |vauthors=van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG |title=Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma |journal=Clin. Cancer Res. |volume=24 |issue=11 |pages=2678–2687 |date=June 2018 |pmid=29511030 |doi=10.1158/1078-0432.CCR-17-3512 |url=}}</ref>
* It may mimic epithelioid [[sarcoma]] on histology but [[metastasis]] is very rare and [[prognosis]] is excellent.<ref name="pmid29406432">{{cite journal |vauthors=Raftopoulos E, Royer M, Warren M, Zhao J, Rush W |title=Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature |journal=Am J Dermatopathol |volume=40 |issue=8 |pages=597–601 |date=August 2018 |pmid=29406432 |doi=10.1097/DAD.0000000000001104 |url=}}</ref>
* It may mimic epithelioid [[sarcoma]] on histology but [[metastasis]] is very rare and [[prognosis]] is excellent.<ref name="pmid29406432">{{cite journal |vauthors=Raftopoulos E, Royer M, Warren M, Zhao J, Rush W |title=Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature |journal=Am J Dermatopathol |volume=40 |issue=8 |pages=597–601 |date=August 2018 |pmid=29406432 |doi=10.1097/DAD.0000000000001104 |url=}}</ref>
* [[Diagnostic]] work up includes [[X-ray]], [[MRI]], [[CT scan]] and [[biopsy]] of the [[lesion]].
* [[Diagnostic]] work up includes [[X-ray]], [[MRI]], [[CT scan]] and [[biopsy]] of the [[lesion]].
* [[Excision]] is the typical treatment but [[chemotherapeutic]] agents including gemcitabine/taxane and mammalian target of rapamycin inhibitor , [[mTOR]] inhibitors such as [[sirolimus]], VEGFR1-4/PDGFRA inhibitors such as telatinib have been used with success in various studies.<ref name="pmid26500758">{{cite journal |vauthors=Joseph J, Wang WL, Patnana M, Ramesh N, Benjamin R, Patel S, Ravi V |title=Cytotoxic and targeted therapy for treatment of pseudomyogenic hemangioendothelioma |journal=Clin Sarcoma Res |volume=5 |issue= |pages=22 |date=2015 |pmid=26500758 |pmc=4615364 |doi=10.1186/s13569-015-0037-8 |url=}}</ref><ref name="pmid28843050">{{cite journal |vauthors=Gabor KM, Sapi Z, Tiszlavicz LG, Fige A, Bereczki C, Bartyik K |title=Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma |journal=Pediatr Blood Cancer |volume=65 |issue=2 |pages= |date=February 2018 |pmid=28843050 |doi=10.1002/pbc.26781 |url=}}</ref><ref name="pmid29511030">{{cite journal |vauthors=van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG |title=Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma |journal=Clin. Cancer Res. |volume=24 |issue=11 |pages=2678–2687 |date=June 2018 |pmid=29511030 |doi=10.1158/1078-0432.CCR-17-3512 |url=}}</ref>
* [[Excision]] is the typical treatment but [[chemotherapeutic]] agents including [[gemcitabine]]/taxane and mammalian target of [[rapamycin]] inhibitor, [[mTOR]] inhibitors such as [[sirolimus]], VEGFR1-4/PDGFRA inhibitors such as telatinib have been used with success in various studies.<ref name="pmid26500758">{{cite journal |vauthors=Joseph J, Wang WL, Patnana M, Ramesh N, Benjamin R, Patel S, Ravi V |title=Cytotoxic and targeted therapy for treatment of pseudomyogenic hemangioendothelioma |journal=Clin Sarcoma Res |volume=5 |issue= |pages=22 |date=2015 |pmid=26500758 |pmc=4615364 |doi=10.1186/s13569-015-0037-8 |url=}}</ref><ref name="pmid28843050">{{cite journal |vauthors=Gabor KM, Sapi Z, Tiszlavicz LG, Fige A, Bereczki C, Bartyik K |title=Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma |journal=Pediatr Blood Cancer |volume=65 |issue=2 |pages= |date=February 2018 |pmid=28843050 |doi=10.1002/pbc.26781 |url=}}</ref><ref name="pmid29511030">{{cite journal |vauthors=van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG |title=Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma |journal=Clin. Cancer Res. |volume=24 |issue=11 |pages=2678–2687 |date=June 2018 |pmid=29511030 |doi=10.1158/1078-0432.CCR-17-3512 |url=}}</ref>


===Polymorphous hemangioendothelioma===
===Polymorphous hemangioendothelioma===
* A rare [[vascular]] [[neoplasm]], Polymorphous hemangioendothelioma occurs in [[lymph nodes]], but a few cases have been found in extra-nodal sites such as the [[mediastinum]], [[spinal cord]], and [[liver]]. It is a very rare cause of persistent [[lymphadenopathy]]. The data on [[natural history]] and clinical presentation is limited due to very few number of cases reported. <ref name="pmid27913780">{{cite journal |vauthors=El Hussein S, Omarzai Y |title=Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature |journal=Int. J. Surg. Pathol. |volume=25 |issue=3 |pages=266–270 |date=May 2017 |pmid=27913780 |doi=10.1177/1066896916679517 |url=}}</ref>
* A rare [[vascular]] [[neoplasm]], polymorphous hemangioendothelioma occurs in [[lymph nodes]], but a few cases have been found in extra-nodal sites such as the [[mediastinum]], [[spinal cord]], and [[liver]]. It is a very rare cause of persistent [[lymphadenopathy]]. The data on [[natural history]] and clinical presentation is limited due to very few number of cases reported.<ref name="pmid27913780">{{cite journal |vauthors=El Hussein S, Omarzai Y |title=Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature |journal=Int. J. Surg. Pathol. |volume=25 |issue=3 |pages=266–270 |date=May 2017 |pmid=27913780 |doi=10.1177/1066896916679517 |url=}}</ref>
* Characterized by a polymorphous blend of solid, primitive [[vascular]] and angiomatous areas in varied proportions on microscopic examination.<ref name="pmid27913780">{{cite journal |vauthors=El Hussein S, Omarzai Y |title=Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature |journal=Int. J. Surg. Pathol. |volume=25 |issue=3 |pages=266–270 |date=May 2017 |pmid=27913780 |doi=10.1177/1066896916679517 |url=}}</ref>
* Characterized by a polymorphous blend of solid, primitive [[vascular]] and angiomatous areas in varied proportions on microscopic examination.<ref name="pmid27913780">{{cite journal |vauthors=El Hussein S, Omarzai Y |title=Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature |journal=Int. J. Surg. Pathol. |volume=25 |issue=3 |pages=266–270 |date=May 2017 |pmid=27913780 |doi=10.1177/1066896916679517 |url=}}</ref>
* [[Diagnotic]] work up includes histopathological examination, [[MRI]] and [[CT scan]].
* [[Diagnotic]] work up includes histopathological examination, [[MRI]], and [[CT scan]].
* Wide local excisio has been used for treatment, with [[radiation]] therapy in case of [[recurrence]].n<ref name="pmid12808568">{{cite journal |vauthors=Tadros M, Rizk SS, Opher E, Thompson LD |title=Polymorphous hemangioendothelioma of the neck |journal=Ann Diagn Pathol |volume=7 |issue=3 |pages=165–8 |date=June 2003 |pmid=12808568 |doi= |url=}}</ref><ref name="pmid19366064">{{cite journal |vauthors=Falleti J, Siano M, De Cecio R, Somma A, Pettinato G, Insabato L |title=Nodal and extranodal soft tissue polymorphous hemangioendothelioma: a case report and review of the literature |journal=Tumori |volume=95 |issue=1 |pages=94–7 |date=2009 |pmid=19366064 |doi= |url=}}</ref>
* Wide local excision has been used for treatment, with [[radiation]] therapy in case of [[recurrence]].<ref name="pmid12808568">{{cite journal |vauthors=Tadros M, Rizk SS, Opher E, Thompson LD |title=Polymorphous hemangioendothelioma of the neck |journal=Ann Diagn Pathol |volume=7 |issue=3 |pages=165–8 |date=June 2003 |pmid=12808568 |doi= |url=}}</ref><ref name="pmid19366064">{{cite journal |vauthors=Falleti J, Siano M, De Cecio R, Somma A, Pettinato G, Insabato L |title=Nodal and extranodal soft tissue polymorphous hemangioendothelioma: a case report and review of the literature |journal=Tumori |volume=95 |issue=1 |pages=94–7 |date=2009 |pmid=19366064 |doi= |url=}}</ref>


===Kaposi sarcoma===
===Kaposi sarcoma===
* An [[AIDS]]-associated [[vascular]] [[malignancy]] that usually presents as [[mucocutaneous]] [[lesions]] <ref name="pmid30191128">{{cite journal |vauthors=Khan S, Guevara J, Barbosa A, Ayuby A, Bien-Aime F, Verda L, Glick N, Mehta V |title=Primary pulmonary Kaposi Sarcoma in a newly diagnosed cisgender heterosexual HIV positive patient presenting before cutaneous manifestations |journal=IDCases |volume=14 |issue= |pages=e00420 |date=2018 |pmid=30191128 |pmc=6125769 |doi=10.1016/j.idcr.2018.e00420 |url=}}</ref> but can also occur in viscera such as lungs. It can remain confined to skin but widespread visceral involvement may occur.
* An [[AIDS]]-associated [[vascular]] [[malignancy]] that usually presents as [[mucocutaneous]] [[lesions]] but can also occur in viscera such as lungs. It can remain confined to skin but widespread visceral involvement may occur.<ref name="pmid30191128">{{cite journal |vauthors=Khan S, Guevara J, Barbosa A, Ayuby A, Bien-Aime F, Verda L, Glick N, Mehta V |title=Primary pulmonary Kaposi Sarcoma in a newly diagnosed cisgender heterosexual HIV positive patient presenting before cutaneous manifestations |journal=IDCases |volume=14 |issue= |pages=e00420 |date=2018 |pmid=30191128 |pmc=6125769 |doi=10.1016/j.idcr.2018.e00420 |url=}}</ref>  
* There are three known variants
* There are three known variants:
** One variant occurs spontaneously in Jewish and Italian males in Europe and the United States.
** One variant occurs spontaneously in Jewish and Italian males in Europe and the United States.
** Another more aggressive variant is endemic in young children is endemic in Africa.  
** Another more aggressive variant is endemic in young children is endemic in Africa.  
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=Malignant vascular tumors=
=Malignant vascular tumors=
===Angiosarcoma===
===Angiosarcoma===
* [[Angiosarcoma]](AS) is [[malignancy]] that presents with a very heterogeneous distribution in the human [[body]] with aggressive clinical course, and may appear in multiple locations, from [[breast]] to [[liver]] or [[skin]].<ref name="pmid30217704">{{cite journal |vauthors=Villaescusa Catalan JM, Martín IG, Cagigal Cobo ML |title=Popliteal Angiosarcoma After Bypass With Autologous Saphenous Vein |journal=Ann Vasc Surg |volume= |issue= |pages= |date=September 2018 |pmid=30217704 |doi=10.1016/j.avsg.2018.06.034 |url=}}</ref>
* [[Angiosarcoma]] (AS) is [[malignancy]] that presents with a very heterogeneous distribution in the human [[body]] with aggressive clinical course, and may appear in multiple locations, from [[breast]] to [[liver]] or [[skin]].<ref name="pmid30217704">{{cite journal |vauthors=Villaescusa Catalan JM, Martín IG, Cagigal Cobo ML |title=Popliteal Angiosarcoma After Bypass With Autologous Saphenous Vein |journal=Ann Vasc Surg |volume= |issue= |pages= |date=September 2018 |pmid=30217704 |doi=10.1016/j.avsg.2018.06.034 |url=}}</ref>
* Associated with MYC gene amplification and [[protein]] overexpression. Myc is a family of regulator genes and [[proto-oncogenes]] that code for [[transcription factors]].<ref name="pmid27780597">{{cite journal |vauthors=Udager AM, Ishikawa MK, Lucas DR, McHugh JB, Patel RM |title=MYC immunohistochemistry in angiosarcoma and atypical vascular lesions: practical considerations based on a single institutional experience |journal=Pathology |volume=48 |issue=7 |pages=697–704 |date=December 2016 |pmid=27780597 |doi=10.1016/j.pathol.2016.08.007 |url=}}</ref>
* Associated with MYC gene amplification and [[protein]] overexpression. Myc is a family of regulator genes and [[proto-oncogenes]] that code for [[transcription factors]].<ref name="pmid27780597">{{cite journal |vauthors=Udager AM, Ishikawa MK, Lucas DR, McHugh JB, Patel RM |title=MYC immunohistochemistry in angiosarcoma and atypical vascular lesions: practical considerations based on a single institutional experience |journal=Pathology |volume=48 |issue=7 |pages=697–704 |date=December 2016 |pmid=27780597 |doi=10.1016/j.pathol.2016.08.007 |url=}}</ref>
* Complete [[surgical excision]] and [[radiotherapy]] are the main treatments, with a minor role of [[chemotherapy]].<ref name="pmid30179666">{{cite journal |vauthors=Priyakumari T, Chandar R, Jayasree K, Ramachandran K |title=Pediatric Primary Ovarian Angiosarcoma: From rarity to a realization |journal=J Pediatr Adolesc Gynecol |volume= |issue= |pages= |date=September 2018 |pmid=30179666 |doi=10.1016/j.jpag.2018.08.008 |url=}}</ref>
* Complete [[surgical excision]] and [[radiotherapy]] are the main treatments, with a minor role of [[chemotherapy]].<ref name="pmid30179666">{{cite journal |vauthors=Priyakumari T, Chandar R, Jayasree K, Ramachandran K |title=Pediatric Primary Ovarian Angiosarcoma: From rarity to a realization |journal=J Pediatr Adolesc Gynecol |volume= |issue= |pages= |date=September 2018 |pmid=30179666 |doi=10.1016/j.jpag.2018.08.008 |url=}}</ref>
For more information about [[angiosarcoma]], [[Angiosarcoma#Angiosarcoma|Click here]].
For more information about [[angiosarcoma]], [[Angiosarcoma|click here]].


===Epithelioid hemangioendothelioma===
===Epithelioid hemangioendothelioma===
* A rare [[vascular]] [[tumor]], described for the first time in 1975 by Dail and Liebow,that usually affects [[lung]], [[liver]] and [[bones]], although may occur many other sites in [[body]] including [[head and neck]], [[breasts]] and [[lymph nodes]].<ref name="pmid25992243">{{cite journal |vauthors=Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M |title=Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor |journal=Oncol Rev |volume=8 |issue=2 |pages=259 |date=September 2014 |pmid=25992243 |pmc=4419652 |doi=10.4081/oncol.2014.259 |url=}}</ref>  
* A rare [[vascular]] [[tumor]], described for the first time in 1975 by Dail and Liebow, that usually affects [[lung]], [[liver]] and [[bones]], although may occur many other sites in [[body]] including [[head and neck]], [[breasts]] and [[lymph nodes]].<ref name="pmid25992243">{{cite journal |vauthors=Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M |title=Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor |journal=Oncol Rev |volume=8 |issue=2 |pages=259 |date=September 2014 |pmid=25992243 |pmc=4419652 |doi=10.4081/oncol.2014.259 |url=}}</ref>  
* Usually [[asymptomatic]] but patient may present with [[respiratory]] symptoms, [[bone]] pains or other symptoms depending on the site of the [[tumor]].
* Usually [[asymptomatic]] but patient may present with [[respiratory]] symptoms, [[bone]] pains or other symptoms depending on the site of the [[tumor]].
* Majority are characterized by a reciprocal t(1;3)(p36;q25) [[translocation]]. The t(1;3) results in fusion of a [[gene]] known as WWTR1 (or TAZ) to CAMTA1. These [[genes code]] for [[transcription factors]].<ref name="pmid21584898">{{cite journal |vauthors=Errani C, Zhang L, Sung YS, Hajdu M, Singer S, Maki RG, Healey JH, Antonescu CR |title=A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites |journal=Genes Chromosomes Cancer |volume=50 |issue=8 |pages=644–53 |date=August 2011 |pmid=21584898 |pmc=3264678 |doi=10.1002/gcc.20886 |url=}}</ref><ref name="pmid21885404">{{cite journal |vauthors=Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, Flanagan J, Luo Y, Fenwick K, Natrajan R, Mitsopoulos C, Zvelebil M, Hoch BL, Weiss SW, Debiec-Rychter M, Sciot R, West RB, Lazar AJ, Ashworth A, Reis-Filho JS, Lord CJ, Gerstein MB, Rubin MA, Rubin BP |title=Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma |journal=Sci Transl Med |volume=3 |issue=98 |pages=98ra82 |date=August 2011 |pmid=21885404 |doi=10.1126/scitranslmed.3002409 |url=}}</ref>
* Majority are characterized by a reciprocal t(1;3)(p36;q25) [[translocation]]. The t(1;3) results in fusion of a [[gene]] known as WWTR1 (or TAZ) to CAMTA1. These [[genes code]] for [[transcription factors]].<ref name="pmid21584898">{{cite journal |vauthors=Errani C, Zhang L, Sung YS, Hajdu M, Singer S, Maki RG, Healey JH, Antonescu CR |title=A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites |journal=Genes Chromosomes Cancer |volume=50 |issue=8 |pages=644–53 |date=August 2011 |pmid=21584898 |pmc=3264678 |doi=10.1002/gcc.20886 |url=}}</ref><ref name="pmid21885404">{{cite journal |vauthors=Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, Flanagan J, Luo Y, Fenwick K, Natrajan R, Mitsopoulos C, Zvelebil M, Hoch BL, Weiss SW, Debiec-Rychter M, Sciot R, West RB, Lazar AJ, Ashworth A, Reis-Filho JS, Lord CJ, Gerstein MB, Rubin MA, Rubin BP |title=Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma |journal=Sci Transl Med |volume=3 |issue=98 |pages=98ra82 |date=August 2011 |pmid=21885404 |doi=10.1126/scitranslmed.3002409 |url=}}</ref>

Latest revision as of 18:51, 26 October 2018


For information on vascular anomalies, click here.

For information on benign vascular tumors, Click here.

Vascular Tumor

Home

Overview

Classification

Benign Vascular tumor
Locally aggressive or borderline vascular tumors
Malignant vascular tumors

Benign Vascular Tumor Home Page

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hannan Javed, M.D.[2], Anmol Pitliya, M.B.B.S. M.D.[3]

Overview

Vascular tumors are growths (benign or malignant) formed from blood vessels; for example, hemangioma, hemangioendothelioma, Kaposi sarcoma, angiosarcoma. International Society for the Study of Vascular Anomalies (ISSVA) has classified vascular tumors into three main categories depending on their clinical and histological behavior. Some lesions related clinically and/or histologically to vascular tumors have been described in provisionally unclassified vascular anomalies and related lesions.

Classification

 
 
 
 
 
 
 
 
 
 
 
 
 
 
Vascular tumors
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Benign
 
 
 
 
 
Locally aggressive or borderline
 
 
 
 
 
Malignant
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Infantile hemangioma / Hemangioma of infancy
 
 
 
 
 
 
Kaposiform hemangioendothelioma
 
 
 
 
 
 
Angiosarcoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Congenital hemangioma
 
 
 
 
 
 
Retiform hemangioendothelioma
 
 
 
 
 
 
Epithelioid hemangioendothelioma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Tufted angioma
 
 
 
 
 
 
Papillary intralymphatic angioendothelioma (PILA), Dabska tumor
 
 
 
 
 
 
Others
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Spindle-cell hemangioma
 
 
 
 
 
 
Composite hemangioendothelioma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Epithelioid hemangioma
 
 
 
 
 
 
Pseudomyogenic hemangioendothelioma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Pyogenic granuloma (also known as lobular capillary hemangioma)
 
 
 
 
 
 
Polymorphous hemangioendothelioma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Others
Microvenular hemangioma
Anastomosing hemangioma
Glomeruloid hemangioma
Papillary hemangioma
Intravascular papillary endothelial hyperplasia
Cutaneous epithelioid angiomatous nodule
Acquired elastotic hemangioma
Littoral cell hemangioma of the spleen
 
 
 
 
 
 
Hemangioendothelioma not otherwise specified
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
'
Related lesions
Eccrine angiomatous hamartoma
Reactive angioendotheliomatosis
Bacillary angiomatosis
 
 
 
 
 
 
Kaposi sarcoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Others
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 


*congenital hemangioma (rapidly involuting type) and tufted angioma may be associated with thrombocytopenia and/or consumptive coagulopathy in some cases. Many experts consider tufted angioma and kaposiform hemangioendothelioma to be part of a spectrum rather than distinct entities
Adapted from International Society for the Study of Vascular Anomalies[1]

Benign vascular tumors

Benign vascular tumors, are benign growths formed from blood vessels; such as, hemangioma, hemangioendothelioma, Kaposi sarcoma. They exhibit a wide range of clinical manifestations, and may occur as isolated lesions or may occur as manifestation of multi-system syndromes and diseases. Their diagnosis and management depends on their clinical manifestations and coexistent anomalies. International Society for the Study of Vascular Anomalies (ISSVA) has classified these lesions into benign vascular tumors and related lesions.

For more information on benign vascular tumors, click here.

Locally aggressive or borderline vascular tumors

Kaposiform hemangioendothelioma

For more information on Kaposiform hemangioendothelioma, click here.

Retiform hemangioendothelioma

Papillary intralymphatic angioendothelioma (PILA), Dabska tumor

  • First described in 1969 by Dabska, this rare vascular neoplasm generally occurs in soft tissues but can also occur in bone. They usually appear as painless inflammatory irregular or nodular lesions below the skin surface.
  • The distinctive feature on histopathology is the intravascular growth of well-differentiated endothelial cells presenting as a matchstick columnar configuration.[7]
  • They are locally aggressive but rarely metastasize. Locally recurrence after surgery is very common.
  • Diagnostic studies may include histopathological studies, fine needle aspiration, MRI, and CT scan.[8]
  • Wide local excision is the treatment of choice. However any combination of steroids, chemotherapy, radiation therapy, and invasive procedures can be used to treat this tumor.

Composite hemangioendothelioma

Pseudomyogenic hemangioendothelioma

Polymorphous hemangioendothelioma

Kaposi sarcoma

  • An AIDS-associated vascular malignancy that usually presents as mucocutaneous lesions but can also occur in viscera such as lungs. It can remain confined to skin but widespread visceral involvement may occur.[19]
  • There are three known variants:
    • One variant occurs spontaneously in Jewish and Italian males in Europe and the United States.
    • Another more aggressive variant is endemic in young children is endemic in Africa.
    • A third form occurs in about 0.04% of kidney transplant patients. There is also a high incidence in AIDS patients. HHV-8 is the suspected cause.[20]

For more information on Kaposi sarcoma, Click here

Malignant vascular tumors

Angiosarcoma

For more information about angiosarcoma, click here.

Epithelioid hemangioendothelioma

References

  1. "Classification | International Society for the Study of Vascular Anomalies".
  2. Hu PA, Zhou ZR (June 2018). "Clinical and imaging features of Kaposiform Hemangioendothelioma". Br J Radiol. 91 (1086): 20170798. doi:10.1259/bjr.20170798. PMID 29536769.
  3. 3.0 3.1 "Letter: Prevention of coronary heart-disease". Lancet. 2 (7980): 313–4. August 1976. PMID 59885.
  4. Lim YH, Bacchiocchi A, Qiu J, Straub R, Bruckner A, Bercovitch L, Narayan D, McNiff J, Ko C, Robinson-Bostom L, Antaya R, Halaban R, Choate KA (August 2016). "GNA14 Somatic Mutation Causes Congenital and Sporadic Vascular Tumors by MAPK Activation". Am. J. Hum. Genet. 99 (2): 443–50. doi:10.1016/j.ajhg.2016.06.010. PMC 4974082. PMID 27476652.
  5. Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Häberle B (2018). "Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options". World J Pediatr. 14 (4): 322–329. doi:10.1007/s12519-018-0171-5. PMID 30054848.
  6. Ranga SM, Kuchangi NC, Shankar VS, Amita K, Haleuoor BB, Belagola SD (November 2014). "Retiform hemangioendothelioma: an uncommon pediatric vascular neoplasm". Indian J Dermatol. 59 (6): 633. doi:10.4103/0019-5154.143583. PMC 4248535. PMID 25484427.
  7. © 1999 Lippincott Williams & Wilkins, Inc.
  8. https://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/
  9. 9.0 9.1 9.2 Rokni GR, Montazer F, Sharifian M, Goldust M (December 2017). "Composite hemangioendothelioma of the forehead and right eye; a case report". BMC Dermatol. 17 (1): 15. doi:10.1186/s12895-017-0067-4. PMC 5727897. PMID 29233122.
  10. Shang Leen SL, Fisher C, Thway K (July 2015). "Composite hemangioendothelioma: clinical and histologic features of an enigmatic entity". Adv Anat Pathol. 22 (4): 254–9. doi:10.1097/PAP.0000000000000079. PMID 26050262.
  11. https://www.jpatholtm.org/upload/pdf/kjp-40-2-142.pdf
  12. 12.0 12.1 12.2 van IJzendoorn D, Sleijfer S, Gelderblom H, Eskens F, van Leenders G, Szuhai K, Bovée J (June 2018). "Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma". Clin. Cancer Res. 24 (11): 2678–2687. doi:10.1158/1078-0432.CCR-17-3512. PMID 29511030. Vancouver style error: initials (help)
  13. 13.0 13.1 Raftopoulos E, Royer M, Warren M, Zhao J, Rush W (August 2018). "Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature". Am J Dermatopathol. 40 (8): 597–601. doi:10.1097/DAD.0000000000001104. PMID 29406432.
  14. Joseph J, Wang WL, Patnana M, Ramesh N, Benjamin R, Patel S, Ravi V (2015). "Cytotoxic and targeted therapy for treatment of pseudomyogenic hemangioendothelioma". Clin Sarcoma Res. 5: 22. doi:10.1186/s13569-015-0037-8. PMC 4615364. PMID 26500758.
  15. Gabor KM, Sapi Z, Tiszlavicz LG, Fige A, Bereczki C, Bartyik K (February 2018). "Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma". Pediatr Blood Cancer. 65 (2). doi:10.1002/pbc.26781. PMID 28843050.
  16. 16.0 16.1 El Hussein S, Omarzai Y (May 2017). "Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature". Int. J. Surg. Pathol. 25 (3): 266–270. doi:10.1177/1066896916679517. PMID 27913780.
  17. Tadros M, Rizk SS, Opher E, Thompson LD (June 2003). "Polymorphous hemangioendothelioma of the neck". Ann Diagn Pathol. 7 (3): 165–8. PMID 12808568.
  18. Falleti J, Siano M, De Cecio R, Somma A, Pettinato G, Insabato L (2009). "Nodal and extranodal soft tissue polymorphous hemangioendothelioma: a case report and review of the literature". Tumori. 95 (1): 94–7. PMID 19366064.
  19. Khan S, Guevara J, Barbosa A, Ayuby A, Bien-Aime F, Verda L, Glick N, Mehta V (2018). "Primary pulmonary Kaposi Sarcoma in a newly diagnosed cisgender heterosexual HIV positive patient presenting before cutaneous manifestations". IDCases. 14: e00420. doi:10.1016/j.idcr.2018.e00420. PMC 6125769. PMID 30191128.
  20. Piccolo V, Russo T, Moscarella E, Brancaccio G, Alfano R, Argenziano G (October 2018). "Dermatoscopy of Vascular Lesions". Dermatol Clin. 36 (4): 389–395. doi:10.1016/j.det.2018.05.006. PMID 30201148.
  21. Villaescusa Catalan JM, Martín IG, Cagigal Cobo ML (September 2018). "Popliteal Angiosarcoma After Bypass With Autologous Saphenous Vein". Ann Vasc Surg. doi:10.1016/j.avsg.2018.06.034. PMID 30217704.
  22. Udager AM, Ishikawa MK, Lucas DR, McHugh JB, Patel RM (December 2016). "MYC immunohistochemistry in angiosarcoma and atypical vascular lesions: practical considerations based on a single institutional experience". Pathology. 48 (7): 697–704. doi:10.1016/j.pathol.2016.08.007. PMID 27780597.
  23. Priyakumari T, Chandar R, Jayasree K, Ramachandran K (September 2018). "Pediatric Primary Ovarian Angiosarcoma: From rarity to a realization". J Pediatr Adolesc Gynecol. doi:10.1016/j.jpag.2018.08.008. PMID 30179666.
  24. 24.0 24.1 Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M (September 2014). "Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor". Oncol Rev. 8 (2): 259. doi:10.4081/oncol.2014.259. PMC 4419652. PMID 25992243.
  25. Errani C, Zhang L, Sung YS, Hajdu M, Singer S, Maki RG, Healey JH, Antonescu CR (August 2011). "A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites". Genes Chromosomes Cancer. 50 (8): 644–53. doi:10.1002/gcc.20886. PMC 3264678. PMID 21584898.
  26. Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, Flanagan J, Luo Y, Fenwick K, Natrajan R, Mitsopoulos C, Zvelebil M, Hoch BL, Weiss SW, Debiec-Rychter M, Sciot R, West RB, Lazar AJ, Ashworth A, Reis-Filho JS, Lord CJ, Gerstein MB, Rubin MA, Rubin BP (August 2011). "Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma". Sci Transl Med. 3 (98): 98ra82. doi:10.1126/scitranslmed.3002409. PMID 21885404.

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