Vascular tumor: Difference between revisions

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__NOTOC__
__NOTOC__
{{Vascular tumor}}
{{Vascular tumor}}
{{CMG}}; {{AE}} {{HMHJ}}
{{CMG}}; {{AE}} {{HMHJ}}
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<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
<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


===Benign vascular tumors===
=Benign vascular tumors=
For details about benign vascular tumors, [[Benign vascular tumor#Benign vascular tumor|Click here]].
For details about benign vascular tumors, [[Benign vascular tumor#Benign vascular tumor|Click here]].


===Locally aggressive or borderline vascular tumors===
=Locally aggressive or borderline vascular tumors=
====Kaposiform hemangioendothelioma====
===Kaposiform hemangioendothelioma===
* Locally Aggressive [[tumor]] that originates on [[skin]] and occurs primarily in [[childhood]].<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> It is characterized by a single or multiple masses with following characteristics:
* Locally Aggressive [[tumor]] that originates on [[skin]] and occurs primarily in [[childhood]].<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> It is characterized by a single or multiple masses with following characteristics:
** Deep reddish-purple color
** Deep reddish-purple color
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* 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>


====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]].
* Must be differentiated from [[Angiosarcoma]].
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* [[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. <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>
* [[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. <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>


====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>
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* 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.


====Composite hemangioendothelioma====
===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>
* 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>
* 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>
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* [[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>
* [[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====
===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>
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* [[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>
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* 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 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====
===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]] <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
* There are three known variants
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* To learn more about KS, click here.
* To learn more about KS, click here.


===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>
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* To learn more about [[angiosarcoma]] click here.
* To learn more about [[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]].

Revision as of 16:25, 12 October 2018


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]


Overview

Vascular tumor may mean:

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

Benign vascular tumors

For details about benign vascular tumors, Click here.

Locally aggressive or borderline vascular tumors

Kaposiform hemangioendothelioma

Retiform hemangioendothelioma

Papillary intralymphatic angioendothelioma (PILA), Dabska tumor

Composite hemangioendothelioma

Pseudomyogenic hemangioendothelioma

Polymorphous hemangioendothelioma

Kaposi sarcoma

  • An AIDS-associated vascular malignancy that usually presents as mucocutaneous lesions [18] 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.[19]
  • To learn more about KS, click here.

Malignant vascular tumors

Angiosarcoma

Epithelioid hemangioendothelioma

References

  1. ↑ 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.
  2. ↑ 2.0 2.1 "Letter: Prevention of coronary heart-disease". Lancet. 2 (7980): 313–4. August 1976. PMID 59885.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 5.0 5.1 5.2 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.
  6. ↑ © 1999 Lippincott Williams & Wilkins, Inc.
  7. ↑ https://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/
  8. ↑ 8.0 8.1 8.2 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.
  9. ↑ 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.
  10. ↑ https://www.jpatholtm.org/upload/pdf/kjp-40-2-142.pdf
  11. ↑ 11.0 11.1 11.2 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)
  12. ↑ 12.0 12.1 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.
  13. ↑ 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.
  14. ↑ 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.
  15. ↑ 15.0 15.1 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.
  16. ↑ Tadros M, Rizk SS, Opher E, Thompson LD (June 2003). "Polymorphous hemangioendothelioma of the neck". Ann Diagn Pathol. 7 (3): 165–8. PMID 12808568.
  17. ↑ 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.
  18. ↑ 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.
  19. ↑ 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.
  20. ↑ 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.
  21. ↑ 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.
  22. ↑ 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.
  23. ↑ 23.0 23.1 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.
  24. ↑ 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.
  25. ↑ 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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