View source for Vascular tumor

__NOTOC__

'''For information on vascular anomalies, [[Vascular anomalies|click here]].'''

'''For information on benign vascular tumors, [[Benign vascular tumor#Benign vascular tumor|Click here]].''' 

{{Vascular tumor}}
{{CMG}}; {{AE}} {{HMHJ}}, {{Anmol}}

==Overview==

'''[[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]]'''.

==Classification==


{{Family tree/start}}
{{Family tree | | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | A01= '''[[Vascular tumors]]'''}}
{{Family tree | | | | | | | |,|-|-|-|-|-|-|-|+|-|-|-|-|-|-|-|.| | | | | | | | | }}
{{Family tree | | | | | | | B01 | | | | | | B02 | | | | | | B03 | | | | | | | | B01='''''Benign'''''|B02='''''Locally aggressive or borderline'''''|B03='''''Malignant'''''}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | |!| | | | | | | | | | }}
{{Family tree | | | | | | |)| C01 | | | | |)| D01 | | | | |)| E01 | | | | | | | C01=[[Infantile hemangioma]] / [[Hemangioma of infancy]]|D01=[[Kaposiform hemangioendothelioma]]|E01=[[Angiosarcoma]]}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | |!| | | | | | | | | | }}
{{Family tree | | | | | | |)| C02 | | | | |)| D02 | | | | |)| E02 | | | | | | | C02=[[Congenital hemangioma]]|D02=[[Retiform hemangioendothelioma]]|E02=[[Epithelioid hemangioendothelioma]]}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | |!| | | | | | | | | | }}
{{Family tree | | | | | | |)| C03 | | | | |)| D03 | | | | |`| E03 | | | | | | | C03=[[Tufted angioma]]|D03=[[Papillary intralymphatic angioendothelioma]] ([[PILA]]), [[Dabska tumor]]|E03=Others}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | |)| C04 | | | | |)| D04 | | | | | | | | | | | | | | | C04=[[Spindle-cell hemangioma]]|D04=[[Composite hemangioendothelioma]]}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | |)| C05 | | | | |)| D05 | | | | | | | | | | | | | | | C05=[[Epithelioid hemangioma]]|D05=[[Pseudomyogenic hemangioendothelioma]]}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | |)| C06 | | | | |)| D06 | | | | | | | | | | | | | | | C06=[[Pyogenic granuloma]] (also known as [[lobular capillary hemangioma]])|D06=[[Polymorphous hemangioendothelioma]]}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | |)| C07 | | | | |)| D07 | | | | | | | | | | | | | | | | |C07=<table>
<tr><td>Others</td></tr>
<tr><td>• [[Microvenular hemangioma]]<td></tr>
<tr><td>• [[Anastomosing hemangioma]]<td></tr>
<tr><td>• [[Glomeruloid hemangioma]]<td></tr>
<tr><td>• [[Papillary hemangioma]]<td></tr>
<tr><td>• [[Intravascular papillary endothelial hyperplasia]]<td></tr>
<tr><td>• [[Cutaneous epithelioid angiomatous nodule]]<td></tr>
<tr><td>• [[Acquired elastotic hemangioma]]<td></tr>
<tr><td>• [[Littoral cell hemangioma of the spleen]]<td></tr>
</table> |D07=Hemangioendothelioma not otherwise specified}}
{{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | |`| C08 | | | | |)| D08 | | | | | | | | | | | | | | | |C08=<table>
<tr><td>Related lesions</td></tr>
<tr><td>• [[Eccrine angiomatous hamartoma]]<td></tr>
<tr><td>• [[Reactive angioendotheliomatosis]]<td></tr>
<tr><td>• [[Bacillary angiomatosis]]<td></tr>'
</table> |D08=[[Kaposi sarcoma]]}}
{{Family tree | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | | | | | | | | | | | | |`| D09 | | | | | | | | | | | | | | | D09=Others}}
{{Family tree/end}}


<sup>*</sup>[[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
<br>'''Adapted from International Society for the Study of Vascular Anomalies'''<ref name="urlClassification | International Society for the Study of Vascular Anomalies">{{cite web |url=http://www.issva.org/classification |title=Classification &#124; International Society for the Study of Vascular Anomalies |format= |work= |accessdate=}}</ref>

=Benign vascular tumors=
''' [[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=
===Kaposiform hemangioendothelioma===
* Locally Aggressive [[tumor]] that originates on [[skin]] and occurs primarily in [[childhood]]. It is characterized by a single or multiple masses with following characteristics:<ref name="pmid29536769">{{cite journal |vauthors=Hu PA, Zhou ZR |title=Clinical and imaging features of Kaposiform Hemangioendothelioma |journal=Br J Radiol |volume=91 |issue=1086 |pages=20170798 |date=June 2018 |pmid=29536769 |doi=10.1259/bjr.20170798 |url=}}</ref>
** Deep reddish-purple color
** Shiny, firm texture
** Warm to the touch
** Swollen and painful
* May be complicated by Kasabach-Merritt phenomenon (KMP), characterized by consumption [[coagulopathy]], [[thrombocytopenia]], and [[hemolytic anemia]].<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> Typical features also include low fibrinogen and elevated d-dimers. 
* [[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>
* [[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>
For more information on [[Kaposiform hemangioendothelioma]], [[Kaposiform hemangioendothelioma#Kaposiform hemangioendothelioma|click here]].

===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>
* It must be differentiated from [[angiosarcoma]].
* 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.
* [[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. 

===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.<ref>© 1999 Lippincott Williams & Wilkins, Inc.</ref>
* 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>
* 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===
* A rare [[vascular]] [[neoplasms]], characterized by an admixture of [[benign]], low-grade [[malignant]], and [[malignant]] vascular components, the ratio of each component can vary. They can occur in any age group.<ref name="pmid29233122">{{cite journal |vauthors=Rokni GR, Montazer F, Sharifian M, Goldust M |title=Composite hemangioendothelioma of the forehead and right eye; a case report |journal=BMC Dermatol. |volume=17 |issue=1 |pages=15 |date=December 2017 |pmid=29233122 |pmc=5727897 |doi=10.1186/s12895-017-0067-4 |url=}}</ref>
* They occur predominantly as long-standing [[lesions]] in the [[dermis]] and [[subcutis]] of the [[extremities]], but can also occur at other sites, including the oral cavity and in viscera such as [[kidney]] and [[spleen]].<ref name="pmid26050262">{{cite journal |vauthors=Shang Leen SL, Fisher C, Thway K |title=Composite hemangioendothelioma: clinical and histologic features of an enigmatic entity |journal=Adv Anat Pathol |volume=22 |issue=4 |pages=254–9 |date=July 2015 |pmid=26050262 |doi=10.1097/PAP.0000000000000079 |url=}}</ref>
* It may recur locally and has the potential to [[metastasize]]. [[Recurrence]] was found to be in 8/10 cases in some studies. <ref>https://www.jpatholtm.org/upload/pdf/kjp-40-2-142.pdf</ref>
* [[Diagnostic]] work up must include [[biopsy]] because of heterogeneity of [[lesions]] and it must be differentiated from other vascular [[tumors]].<ref name="pmid29233122">{{cite journal |vauthors=Rokni GR, Montazer F, Sharifian M, Goldust M |title=Composite hemangioendothelioma of the forehead and right eye; a case report |journal=BMC Dermatol. |volume=17 |issue=1 |pages=15 |date=December 2017 |pmid=29233122 |pmc=5727897 |doi=10.1186/s12895-017-0067-4 |url=}}</ref>
* [[Surgical]] excision is the treatment of choice although some patients have been treated with interferons and electron beams.<ref name="pmid29233122">{{cite journal |vauthors=Rokni GR, Montazer F, Sharifian M, Goldust M |title=Composite hemangioendothelioma of the forehead and right eye; a case report |journal=BMC Dermatol. |volume=17 |issue=1 |pages=15 |date=December 2017 |pmid=29233122 |pmc=5727897 |doi=10.1186/s12895-017-0067-4 |url=}}</ref>

===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>
* 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>
* [[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>

===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>
* 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]].
* 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>

===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. 
* 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.<ref name="pmid30201148">{{cite journal |vauthors=Piccolo V, Russo T, Moscarella E, Brancaccio G, Alfano R, Argenziano G |title=Dermatoscopy of Vascular Lesions |journal=Dermatol Clin |volume=36 |issue=4 |pages=389–395 |date=October 2018 |pmid=30201148 |doi=10.1016/j.det.2018.05.006 |url=}}</ref>
For more information on [[Kaposi sarcoma]], [[Kaposi's sarcoma#Kaposi's sarcoma|Click here]]

=Malignant vascular tumors=
===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>
* 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>
For more information about [[angiosarcoma]], [[Angiosarcoma#Angiosarcoma|Click here]].

===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> 
* 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>
* [[Imaging]] is crucial in forming both [[diagnosis]] and management plan. Recognition of the expression of [[vascular]] markers (Fli-1 and CD31 are endothelial-specific markers), and the microscopic evidence of vascular differentiation is of primary importance as well.<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>
* [[Surgery]] has been used as primary treatment modality depending upon the location of the [[tumor]], with [[radiotherapy]] being used in some cases.

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