Vascular tumor: Difference between revisions

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'''Vascular tumor''' may mean:
__NOTOC__


:* '''[[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.
'''For information on vascular anomalies, [[Vascular anomalies|click here]].'''
:* a '''highly vascularized tumor''' or '''poorly vascularized tumor''', referring to the degree of adequate or inadequate ([[ischemic]])  blood supply to  a tumor formed from any [[biological tissue]]


==Vascular Tumors==
'''For information on benign vascular tumors, [[Benign vascular tumor#Benign vascular tumor|Click here]].'''
===Benign vascular tumors 1===
====Infantile hemangioma / Hemangioma of infancy====
{| class="wikitable"                                     
|+                                                       
!Pattern                                                 
|-                                                       
|
* focal             


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


* segmental
==Overview==


* indeterminate
'''[[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]]'''.
|}
{| class="wikitable"
|+
!Different types
|-
|
* superficial


* deep
==Classification==


* mixed (superficial + deep)


* reticular / abortive / minimal growth
{{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}}


* others
|}
{| class="wikitable"
|+
! colspan="2" |Association with other lesions
|-
|PHACE association /
syndrome
| rowspan="2" |Posterior fossa malformations, Hemangioma, Arterial
anomalies, Cardiovascular anomalies, Eye anomalies ,


sternal clefting and ⁄ or supraumbilical raphe
<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>
| rowspan="2" |LUMBAR (SACRAL,
PELVIS) association /


syndrome
=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]].
|Lower body hemangioma, Urogenital anomalies,
Ulceration, Myelopathy, Bony deformities, Anorectal


malformations, Arterial anomalies, and Renal anomalies
For more information on benign vascular tumors, [[Benign vascular tumor#Benign vascular tumor|click here]].
|}
* Most common tumor of infancy. Usually appearing after birth, infantile hemangiomas undergo a period of proliferation in few weeks after birth followed by regression and involution in first year of life. Superficial lesions appear as red or “strawberry” colored nodules, papules, or plaques while deeper hemangiomas are typically bluish or skin colored. Mixed tumors, involving both epidermis and deeper structures, may display characteristics of both. They may also be classified as focal, that appear in a specific anatomical area, and segmental that shows varied pattern of growth following developmental growth regions. Segmental type is often associated with other developmental abnormalities.<ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid12472344">{{cite journal |vauthors=Chiller KG, Passaro D, Frieden IJ |title=Hemangiomas of infancy: clinical characteristics, morphologic subtypes, and their relationship to race, ethnicity, and sex |journal=Arch Dermatol |volume=138 |issue=12 |pages=1567–76 |date=December 2002 |pmid=12472344 |doi= |url=}}</ref><ref name="pmid22229118">{{cite journal |vauthors=Greenberger S, Bischoff J |title=Infantile hemangioma-mechanism(s) of drug action on a vascular tumor |journal=Cold Spring Harb Perspect Med |volume=1 |issue=1 |pages=a006460 |date=September 2011 |pmid=22229118 |pmc=3234458 |doi=10.1101/cshperspect.a006460 |url=}}</ref>
* Rarely, infantile hemangioms can cause life-threatening complications such as congestive cardiac failure, respiratory difficulty and respiratory compromise, and loss of vision. There may also be long-term sequela including permanent disfigurement and scarring. If lesions are multiple, there is an increased risk of visceral involvement. There may be an association with certain syndromes such as PHACE syndrome.<ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid29924216">{{cite journal |vauthors=Rotter A, Samorano LP, Rivitti-Machado MC, Oliveira ZNP, Gontijo B |title=PHACE syndrome: clinical manifestations, diagnostic criteria, and management |journal=An Bras Dermatol |volume=93 |issue=3 |pages=405–411 |date=June 2018 |pmid=29924216 |pmc=6001075 |doi=10.1590/abd1806-4841.20187693 |url=}}</ref>
* Some studies have indicated autosomal-dominant and maternal patterns of inheritance. Some studies suggest that environmental factors play the key role. Some risk factors that have been identified in association with infantile hemangioma include female gender, preterm birth, low weight at birth, increasing maternal age ta birth, placenta previa, pre-eclampsia, progesterone use by mother, and Caucasian race.<ref name="pmid27940781">{{cite journal |vauthors=Castrén E, Salminen P, Vikkula M, Pitkäranta A, Klockars T |title=Inheritance Patterns of Infantile Hemangioma |journal=Pediatrics |volume=138 |issue=5 |pages= |date=November 2016 |pmid=27940781 |doi=10.1542/peds.2016-1623 |url=}}</ref><ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid17307549">{{cite journal |vauthors=Haggstrom AN, Drolet BA, Baselga E, Chamlin SL, Garzon MC, Horii KA, Lucky AW, Mancini AJ, Metry DW, Newell B, Nopper AJ, Frieden IJ |title=Prospective study of infantile hemangiomas: demographic, prenatal, and perinatal characteristics |journal=J. Pediatr. |volume=150 |issue=3 |pages=291–4 |date=March 2007 |pmid=17307549 |doi=10.1016/j.jpeds.2006.12.003 |url=}}</ref><ref name="pmid18940356">{{cite journal |vauthors=Drolet BA, Swanson EA, Frieden IJ |title=Infantile hemangiomas: an emerging health issue linked to an increased rate of low birth weight infants |journal=J. Pediatr. |volume=153 |issue=5 |pages=712–5, 715.e1 |date=November 2008 |pmid=18940356 |doi=10.1016/j.jpeds.2008.05.043 |url=}}</ref>
* THE diagnosis is made clinically and a thorough investigation should be carried out for visceral hemangiomas and other associative abnormalities if suspicion arises. Majority of these lesions do not require any treatment but treatment is indicated if there is risk for complications such as visual or respiratory involvement. Elective treatment is also offered to prevent disfigurement or scarring. Recently there have been an increased usage of oral beta-blockers such as timolol over systemic glucocorticoids because of higher efficacy.  Vincristine and interferon alpha have been used in some high risk hemangiomas but carry the risk of severe complications. Visceral hemangioms may require embolization or surgery if they do not respond to systemic therapy. Laser therapy especially PDL is another modality used in cases of hemangioms unresponsive to medication.<ref name="pmid28402073">{{cite journal |vauthors=Smith CJF, Friedlander SF, Guma M, Kavanaugh A, Chambers CD |title=Infantile Hemangiomas: An Updated Review on Risk Factors, Pathogenesis, and Treatment |journal=Birth Defects Res |volume=109 |issue=11 |pages=809–815 |date=July 2017 |pmid=28402073 |pmc=5839165 |doi=10.1002/bdr2.1023 |url=}}</ref><ref name="pmid26416928">{{cite journal |vauthors=Darrow DH, Greene AK, Mancini AJ, Nopper AJ |title=Diagnosis and Management of Infantile Hemangioma: Executive Summary |journal=Pediatrics |volume=136 |issue=4 |pages=786–91 |date=October 2015 |pmid=26416928 |doi=10.1542/peds.2015-2482 |url=}}</ref><ref name="pmid14871317">{{cite journal |vauthors=Ceisler EJ, Santos L, Blei F |title=Periocular hemangiomas: what every physician should know |journal=Pediatr Dermatol |volume=21 |issue=1 |pages=1–9 |date=2004 |pmid=14871317 |doi= |url=}}</ref><ref name="pmid26859502">{{cite journal |vauthors=Tal R, Dotan M, Lorber A |title=Approach to haemangiomatosis causing congestive heart failure |journal=Acta Paediatr. |volume=105 |issue=6 |pages=600–4 |date=June 2016 |pmid=26859502 |doi=10.1111/apa.13359 |url=}}</ref><ref name="pmid21788220">{{cite journal |vauthors=Hogeling M, Adams S, Wargon O |title=A randomized controlled trial of propranolol for infantile hemangiomas |journal=Pediatrics |volume=128 |issue=2 |pages=e259–66 |date=August 2011 |pmid=21788220 |doi=10.1542/peds.2010-0029 |url=}}</ref><ref name="pmid11559219">{{cite journal |vauthors=Bennett ML, Fleischer AB, Chamlin SL, Frieden IJ |title=Oral corticosteroid use is effective for cutaneous hemangiomas: an evidence-based evaluation |journal=Arch Dermatol |volume=137 |issue=9 |pages=1208–13 |date=September 2001 |pmid=11559219 |doi= |url=}}</ref><ref name="pmid12102167">{{cite journal |vauthors=Perez J, Pardo J, Gomez C |title=Vincristine--an effective treatment of corticoid-resistant life-threatening infantile hemangiomas |journal=Acta Oncol |volume=41 |issue=2 |pages=197–9 |date=2002 |pmid=12102167 |doi= |url=}}</ref><ref name="pmid20936416">{{cite journal |vauthors=Schiestl C, Neuhaus K, Zoller S, Subotic U, Forster-Kuebler I, Michels R, Balmer C, Weibel L |title=Efficacy and safety of propranolol as first-line treatment for infantile hemangiomas |journal=Eur. J. Pediatr. |volume=170 |issue=4 |pages=493–501 |date=April 2011 |pmid=20936416 |doi=10.1007/s00431-010-1324-2 |url=}}</ref><ref name="pmid26711436">{{cite journal |vauthors=Chinnadurai S, Sathe NA, Surawicz T |title=Laser treatment of infantile hemangioma: A systematic review |journal=Lasers Surg Med |volume=48 |issue=3 |pages=221–33 |date=March 2016 |pmid=26711436 |doi=10.1002/lsm.22455 |url=}}</ref>
* To learn more about infantile hemagioma click here.


====Congenital hemangioma====
=Locally aggressive or borderline vascular tumors=
* Rare tumor that arises in utero and presents as fully developed lesion at birth. Following birth they can regress completely, partially or not at all. So they can be classified as Rapidly involuting (RICH), Non-involuting (NICH), Partially involuting (PICH).<ref name="urlwww.issva.org">{{cite web |url=http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf |title=www.issva.org |format= |work= |accessdate=}}</ref><ref name="pmid27058448">{{cite journal |vauthors=Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HP, Bischoff J, Greene AK, Warman ML |title=Somatic Activating Mutations in GNAQ and GNA11 Are Associated with Congenital Hemangioma |journal=Am. J. Hum. Genet. |volume=98 |issue=4 |pages=789–95 |date=April 2016 |pmid=27058448 |pmc=4833432 |doi=10.1016/j.ajhg.2016.03.009 |url=}}</ref>
===Kaposiform hemangioendothelioma===
*# '''Rapidly involuting (RICH)'''
* 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>
*#* This fast flow tumor can be detected in utero and appears as raised gray single lesions with dilated veins, telangiectasias and a halo at birth. This tumor may be complicated by thrombocytopenia and congestive cardiac failure due to its high-flow nature. Tumor typically regresses spontaneously in 1 to 2 years of life. Sometimes it can occur in liver where it follows the same pattern of involution as that of skin.<ref name="pmid27058448">{{cite journal |vauthors=Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HP, Bischoff J, Greene AK, Warman ML |title=Somatic Activating Mutations in GNAQ and GNA11 Are Associated with Congenital Hemangioma |journal=Am. J. Hum. Genet. |volume=98 |issue=4 |pages=789–95 |date=April 2016 |pmid=27058448 |pmc=4833432 |doi=10.1016/j.ajhg.2016.03.009 |url=}}</ref><ref name="pmid15018449">{{cite journal |vauthors=Berenguer B, Mulliken JB, Enjolras O, Boon LM, Wassef M, Josset P, Burrows PE, Perez-Atayde AR, Kozakewich HP |title=Rapidly involuting congenital hemangioma: clinical and histopathologic features |journal=Pediatr. Dev. Pathol. |volume=6 |issue=6 |pages=495–510 |date=2003 |pmid=15018449 |doi= |url=}}</ref>
*# '''Non-involuting (NICH)'''
*#* Fast flow tumor that presents as well defined, plaque like lesion with pink to purple color, telangiectasias and pale borders. Typically remains stable but there have been some reports of growth and expansion.<ref name="pmid27058448">{{cite journal |vauthors=Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HP, Bischoff J, Greene AK, Warman ML |title=Somatic Activating Mutations in GNAQ and GNA11 Are Associated with Congenital Hemangioma |journal=Am. J. Hum. Genet. |volume=98 |issue=4 |pages=789–95 |date=April 2016 |pmid=27058448 |pmc=4833432 |doi=10.1016/j.ajhg.2016.03.009 |url=}}</ref>
*# '''Partially involuting (PICH)'''
*#* These lesions start involution as RICH but become stable over time and persist as NICH.<ref name="pmid24176519">{{cite journal |vauthors=Nasseri E, Piram M, McCuaig CC, Kokta V, Dubois J, Powell J |title=Partially involuting congenital hemangiomas: a report of 8 cases and review of the literature |journal=J. Am. Acad. Dermatol. |volume=70 |issue=1 |pages=75–9 |date=January 2014 |pmid=24176519 |doi=10.1016/j.jaad.2013.09.018 |url=}}</ref>
* Somatic mutations in GNAQ/GNA11 are thought to cause the congenital hemangioma. GNAQ and its paralogue GNA11 function in intracellular signaling pathways as Gq alpha subunit.<ref name="pmid27058448">{{cite journal |vauthors=Ayturk UM, Couto JA, Hann S, Mulliken JB, Williams KL, Huang AY, Fishman SJ, Boyd TK, Kozakewich HP, Bischoff J, Greene AK, Warman ML |title=Somatic Activating Mutations in GNAQ and GNA11 Are Associated with Congenital Hemangioma |journal=Am. J. Hum. Genet. |volume=98 |issue=4 |pages=789–95 |date=April 2016 |pmid=27058448 |pmc=4833432 |doi=10.1016/j.ajhg.2016.03.009 |url=}}</ref><ref name="urlError 403">{{cite web |url=https://www.omim.org/entry/139313 |title=Error 403 |format= |work= |accessdate=}}</ref>
* Diagnosis is usually clinical but imaging techniques such as MRI, CT scan, contrast-enhanced ultrasound and later biopsy can be considered if required. Surgical excision should be considered in case of complications, NICH and PICH.<ref name="pmid29922526">{{cite journal |vauthors=Ramphul K, Mejias SG, Ramphul-Sicharam Y, Sonaye R |title=Congenital Hemangioma: A Case Report of a Finding Every Physician Should Know |journal=Cureus |volume=10 |issue=4 |pages=e2485 |date=April 2018 |pmid=29922526 |pmc=6003795 |doi=10.7759/cureus.2485 |url=}}</ref><ref name="pmid29971555">{{cite journal |vauthors=Thimm MA, Rhee D, Takemoto CM, Karnsakul W, Cuffari C, Guerrerio AL, Garcia A, Gearhart J, Huisman TAGM, Hwang M |title=Diagnosis of congenital and acquired focal lesions in the neck, abdomen, and pelvis with contrast-enhanced ultrasound: a pictorial essay |journal=Eur. J. Pediatr. |volume=177 |issue=10 |pages=1459–1470 |date=October 2018 |pmid=29971555 |doi=10.1007/s00431-018-3197-8 |url=}}</ref>
====Tufted angioma====
* Benign tumor that is characterized by dense clumps of endothelial cells and capillaries located in dermis. Most lesions appear in adolescence but some can manifest at birth or late in adulthood. Clinical presentation varies but majority of lesions appear as solitary stained area or elevation that later forms red-purple or dusky red plaque while some lesions appear as firm nodules. Lesions are usually solitary, asymptomatic but tender with occasional painful episodes and located on trunk and neck in majority of the cases. Some cases of multi-focal tufted angiomas have also been reported.<ref name="pmid26225332">{{cite journal |vauthors=Prasuna A, Rao PN |title=A tufted angioma |journal=Indian Dermatol Online J |volume=6 |issue=4 |pages=266–8 |date=2015 |pmid=26225332 |pmc=4513407 |doi=10.4103/2229-5178.160259 |url=}}</ref><ref name="pmid2644316">{{cite journal |vauthors=Jones EW, Orkin M |title=Tufted angioma (angioblastoma). A benign progressive angioma, not to be confused with Kaposi's sarcoma or low-grade angiosarcoma |journal=J. Am. Acad. Dermatol. |volume=20 |issue=2 Pt 1 |pages=214–25 |date=February 1989 |pmid=2644316 |doi= |url=}}</ref><ref name="pmid26312663">{{cite journal |vauthors=Pesapane F, Nazzaro G, Alberti-Violetti S, Gianotti R |title=A case of acquired tufted angioma in adulthood |journal=An Bras Dermatol |volume=90 |issue=3 Suppl 1 |pages=16–8 |date=2015 |pmid=26312663 |pmc=4540497 |doi=10.1590/abd1806-4841.20153733 |url=}}</ref><ref name="pmid26288441">{{cite journal |vauthors=Bandyopadhyay D, Saha A |title=Multifocal Annular Tufted Angioma: An Uncommon Clinical Entity |journal=Indian J Dermatol |volume=60 |issue=4 |pages=422 |date=2015 |pmid=26288441 |pmc=4533571 |doi=10.4103/0019-5154.160528 |url=}}</ref>
* Tufted angioma can be associated with Kasabach-Merritt phenomenon.<ref name="pmid26288441">{{cite journal |vauthors=Bandyopadhyay D, Saha A |title=Multifocal Annular Tufted Angioma: An Uncommon Clinical Entity |journal=Indian J Dermatol |volume=60 |issue=4 |pages=422 |date=2015 |pmid=26288441 |pmc=4533571 |doi=10.4103/0019-5154.160528 |url=}}</ref><ref name="urlwww.issva.org">{{cite web |url=http://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf |title=www.issva.org |format= |work= |accessdate=}}</ref><ref name="pmid26225332">{{cite journal |vauthors=Prasuna A, Rao PN |title=A tufted angioma |journal=Indian Dermatol Online J |volume=6 |issue=4 |pages=266–8 |date=2015 |pmid=26225332 |pmc=4513407 |doi=10.4103/2229-5178.160259 |url=}}</ref>
* Somatic activating GNA14 c.614A>T (p.Gln205Leu) mutations have been found in some tufted angiomas. These mutations may cause the cell growth to be growth-factor independent by up-regulating the MAPK pathway.<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><ref name="urlGNA14 G protein subunit alpha 14 [Homo sapiens (human)] - Gene - NCBI">{{cite web |url=https://www.ncbi.nlm.nih.gov/gene/9630 |title=GNA14 G protein subunit alpha 14 [Homo sapiens (human)] - Gene - NCBI |format= |work= |accessdate=}}</ref>
* Imaging such as MRI and ultrasound can add into clinical diagnosis to differentiate tufted angioma from similar lesions such as Kaposi sarcoma, kaposiform hemangioendothelioma and infantile hemangioma. Biopsy and histopathological studies are sometimes required for accurate diagnosis. Surgical excision is the treatment modality but some recommend to only observe the lesions due to its slow growth and possible remission. Other therapies include radiation beam therapy, cryosurgery, corticosteroids and pulsed laser therapy. Vincristine and embolization has been used with success in angiomas associated with Kasabach-Merritt phenomenon.<ref name="pmid26288441">{{cite journal |vauthors=Bandyopadhyay D, Saha A |title=Multifocal Annular Tufted Angioma: An Uncommon Clinical Entity |journal=Indian J Dermatol |volume=60 |issue=4 |pages=422 |date=2015 |pmid=26288441 |pmc=4533571 |doi=10.4103/0019-5154.160528 |url=}}</ref><ref name="pmid21965850">{{cite journal |vauthors=Ghosh SK, Bandyopadhyay D, Ghosh A, Biswas SK, Barma KD |title=Acquired multifocal tufted angiomas in an immunocompetent young adult |journal=Indian J Dermatol |volume=56 |issue=4 |pages=412–4 |date=July 2011 |pmid=21965850 |pmc=3179005 |doi=10.4103/0019-5154.84741 |url=}}</ref><ref name="pmid20644037">{{cite journal |vauthors=Osio A, Fraitag S, Hadj-Rabia S, Bodemer C, de Prost Y, Hamel-Teillac D |title=Clinical spectrum of tufted angiomas in childhood: a report of 13 cases and a review of the literature |journal=Arch Dermatol |volume=146 |issue=7 |pages=758–63 |date=July 2010 |pmid=20644037 |doi=10.1001/archdermatol.2010.135 |url=}}</ref><ref name="pmid17520468">{{cite journal |vauthors=Chiu CS, Yang LC, Hong HS, Kuan YZ |title=Treatment of a tufted angioma with intense pulsed light |journal=J Dermatolog Treat |volume=18 |issue=2 |pages=109–11 |date=2007 |pmid=17520468 |doi=10.1080/09546630601028752 |url=}}</ref><ref name="pmid18301012">{{cite journal |vauthors=Yesudian PD, Parslew R, Klafowski J, Gould D, Pizer B |title=Tufted angioma-associated Kasabach-Merritt syndrome treated with embolization and vincristine |journal=Plast. Reconstr. Surg. |volume=121 |issue=2 |pages=692–3 |date=February 2008 |pmid=18301012 |doi=10.1097/01.prs.0000298541.92722.5d |url=}}</ref><ref name="pmid29166525">{{cite journal |vauthors=Silva CMD, Schettini APM, Santos M, Chirano CAR |title=Tufted angioma |journal=An Bras Dermatol |volume=92 |issue=5 |pages=742–743 |date=2017 |pmid=29166525 |pmc=5674719 |doi=10.1590/abd1806-4841.20175896 |url=}}</ref><ref name="pmid28623854">{{cite journal |vauthors=Kimura R, Yoshida Y, Wakumoto K, Yamada N, Yamamoto O |title=Successful treatment of tufted angioma with low-dose electron beam radiation therapy: Report of two cases |journal=J. Dermatol. |volume=44 |issue=10 |pages=e262–e263 |date=October 2017 |pmid=28623854 |doi=10.1111/1346-8138.13936 |url=}}</ref>
====Spindle-cell hemangioma====
* Rare benign tumor that manifests as solitary or multiple nodules confined to dermis and subcutaneous tissues in almost all of the cases. Histopathologically it appears as solid areas that are cellular and consist of spindle cells seen attached to vessel walls, and cavernous spaces that can be thrombosed. Size may increase over time and patient usually complains of swelling and pain. The nodules or masses can be mobile and elastic or can be firm and immobile.<ref name="pmid29780644">{{cite journal |vauthors=Murakami K, Yamamoto K, Sugiura T, Kirita T |title=Spindle Cell Hemangioma in the Mucosa of the Upper Lip: A Case Report and Review of the Literature |journal=Case Rep Dent |volume=2018 |issue= |pages=1370701 |date=2018 |pmid=29780644 |pmc=5892276 |doi=10.1155/2018/1370701 |url=}}</ref><ref name="pmid26266229">{{cite journal |vauthors=Chavva S, Priya MH, Garlapati K, Reddy GS, Gannepalli A |title=Rare Case of Spindle Cell Haemangioma |journal=J Clin Diagn Res |volume=9 |issue=6 |pages=ZD19–21 |date=June 2015 |pmid=26266229 |pmc=4525619 |doi=10.7860/JCDR/2015/11998.6080 |url=}}</ref><ref name="pmid22606579">{{cite journal |vauthors=Minagawa T, Yamao T, Shioya R |title=Spindle cell hemangioendothelioma of the temporal muscle resected with zygomatic osteotomy: a case report of an unusual intramuscular lesion mimicking sarcoma |journal=Case Rep Surg |volume=2011 |issue= |pages=481654 |date=2011 |pmid=22606579 |pmc=3350060 |doi=10.1155/2011/481654 |url=}}</ref>
* Somatic mutations in IDH1 and IDH2 have been found to be present in 70% of spindle-cell hemangiomas. IDH1 and IDH2 are important enzymes in cell energy cycles (α-ketoglutarate and NADPH generation).<ref name="pmid22057234">{{cite journal |vauthors=Pansuriya TC, van Eijk R, d'Adamo P, van Ruler MA, Kuijjer ML, Oosting J, Cleton-Jansen AM, van Oosterwijk JG, Verbeke SL, Meijer D, van Wezel T, Nord KH, Sangiorgi L, Toker B, Liegl-Atzwanger B, San-Julian M, Sciot R, Limaye N, Kindblom LG, Daugaard S, Godfraind C, Boon LM, Vikkula M, Kurek KC, Szuhai K, French PJ, Bovée JV |title=Somatic mosaic IDH1 and IDH2 mutations are associated with enchondroma and spindle cell hemangioma in Ollier disease and Maffucci syndrome |journal=Nat. Genet. |volume=43 |issue=12 |pages=1256–61 |date=November 2011 |pmid=22057234 |pmc=3427908 |doi=10.1038/ng.1004 |url=}}</ref><ref name="pmid22343901">{{cite journal |vauthors=Lu C, Ward PS, Kapoor GS, Rohle D, Turcan S, Abdel-Wahab O, Edwards CR, Khanin R, Figueroa ME, Melnick A, Wellen KE, O'Rourke DM, Berger SL, Chan TA, Levine RL, Mellinghoff IK, Thompson CB |title=IDH mutation impairs histone demethylation and results in a block to cell differentiation |journal=Nature |volume=483 |issue=7390 |pages=474–8 |date=February 2012 |pmid=22343901 |pmc=3478770 |doi=10.1038/nature10860 |url=}}</ref>
* Diagnosis often requires biopsy and imaging studies such as MRI to ascertain the extent of the tumor. Local excision is the treatment modality of choice with excellent prognosis in majority of the cases although recurrence is very common.<ref name="pmid29780644">{{cite journal |vauthors=Murakami K, Yamamoto K, Sugiura T, Kirita T |title=Spindle Cell Hemangioma in the Mucosa of the Upper Lip: A Case Report and Review of the Literature |journal=Case Rep Dent |volume=2018 |issue= |pages=1370701 |date=2018 |pmid=29780644 |pmc=5892276 |doi=10.1155/2018/1370701 |url=}}</ref><ref name="pmid26266229">{{cite journal |vauthors=Chavva S, Priya MH, Garlapati K, Reddy GS, Gannepalli A |title=Rare Case of Spindle Cell Haemangioma |journal=J Clin Diagn Res |volume=9 |issue=6 |pages=ZD19–21 |date=June 2015 |pmid=26266229 |pmc=4525619 |doi=10.7860/JCDR/2015/11998.6080 |url=}}</ref><ref name="pmid22606579">{{cite journal |vauthors=Minagawa T, Yamao T, Shioya R |title=Spindle cell hemangioendothelioma of the temporal muscle resected with zygomatic osteotomy: a case report of an unusual intramuscular lesion mimicking sarcoma |journal=Case Rep Surg |volume=2011 |issue= |pages=481654 |date=2011 |pmid=22606579 |pmc=3350060 |doi=10.1155/2011/481654 |url=}}</ref><ref name="pmid23730241">{{cite journal |vauthors=Gray SS, Eltorky MA, Riascos RF, Montilla RD |title=Spindle cell hemangioma reoccurrence in the hand: case report |journal=Hand (N Y) |volume=7 |issue=2 |pages=194–9 |date=June 2012 |pmid=23730241 |pmc=3351523 |doi=10.1007/s11552-012-9397-1 |url=}}</ref><ref name="pmid8827025">{{cite journal |vauthors=Perkins P, Weiss SW |title=Spindle cell hemangioendothelioma. An analysis of 78 cases with reassessment of its pathogenesis and biologic behavior |journal=Am. J. Surg. Pathol. |volume=20 |issue=10 |pages=1196–204 |date=October 1996 |pmid=8827025 |doi= |url=}}</ref>
====Epithelioid hemangioma====
* Rare benign tumor that typically presents as solitary painful nodule on head and neck. Penis is an atypical location. They can involve skin, bone and soft tissues. Histopathologically these lesions are characterized by presence of endothelial cells that resemble epithelial cells with evidence of proliferation such as large nuclei and prominent nucleoli, and often inflammatory infiltrates. Vessels are typically well-formed. Nuclear atypia is absent.<ref name="pmid25295139">{{cite journal |vauthors=Barber E, Domes T |title=Painful erections secondary to rare epithelioid hemangioma of the penis |journal=Can Urol Assoc J |volume=8 |issue=9-10 |pages=E647–9 |date=September 2014 |pmid=25295139 |pmc=4164556 |doi=10.5489/cuaj.1833 |url=}}</ref><ref name="pmid28159479">{{cite journal |vauthors=Tsikopoulos K, Perdikakis E, Georgiannos D, Bisbinas I |title=Epithelioid hemangioma of the scapula treated with chemoembolization and microwave ablation: Α case report |journal=Acta Orthop Traumatol Turc |volume=52 |issue=2 |pages=157–161 |date=March 2018 |pmid=28159479 |pmc=6136344 |doi=10.1016/j.aott.2017.01.003 |url=}}</ref><ref name="pmid24314244">{{cite journal |vauthors=Gong QX, Fan QH, Xie J, Su ZL, Zhang MH, Zhang ZH |title=[Epithelioid hemangioma: a clinicopathologic analysis of 7 cases] |language=Chinese |journal=Zhonghua Bing Li Xue Za Zhi |volume=42 |issue=9 |pages=593–8 |date=September 2013 |pmid=24314244 |doi= |url=}}</ref>
* FOS gene rearrangements such as  ZFP36-FOSB fusions are found to be present in one third of epithelioid hemangioma in a study. It encodes a transcription factors that causes over-expression of vascular endothelial growth factor-D (VEGF-D).<ref name="pmid16199154">{{cite journal |vauthors=Milde-Langosch K |title=The Fos family of transcription factors and their role in tumourigenesis |journal=Eur. J. Cancer |volume=41 |issue=16 |pages=2449–61 |date=November 2005 |pmid=16199154 |doi=10.1016/j.ejca.2005.08.008 |url=}}</ref><ref name="pmid19760594">{{cite journal |vauthors=Durchdewald M, Angel P, Hess J |title=The transcription factor Fos: a Janus-type regulator in health and disease |journal=Histol. Histopathol. |volume=24 |issue=11 |pages=1451–61 |date=November 2009 |pmid=19760594 |doi=10.14670/HH-24.1451 |url=}}</ref><ref name="pmid10449752">{{cite journal |vauthors=Marconcini L, Marchio S, Morbidelli L, Cartocci E, Albini A, Ziche M, Bussolino F, Oliviero S |title=c-fos-induced growth factor/vascular endothelial growth factor D induces angiogenesis in vivo and in vitro |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=96 |issue=17 |pages=9671–6 |date=August 1999 |pmid=10449752 |pmc=22268 |doi= |url=}}</ref><ref name="pmid26135557">{{cite journal |vauthors=Huang SC, Zhang L, Sung YS, Chen CL, Krausz T, Dickson BC, Kao YC, Agaram NP, Fletcher CD, Antonescu CR |title=Frequent FOS Gene Rearrangements in Epithelioid Hemangioma: A Molecular Study of 58 Cases With Morphologic Reappraisal |journal=Am. J. Surg. Pathol. |volume=39 |issue=10 |pages=1313–21 |date=October 2015 |pmid=26135557 |pmc=4567921 |doi=10.1097/PAS.0000000000000469 |url=}}</ref>
* Diagnosis requires biopsy to determine characteristic histopathological features. Imaging techniques such as MRI can useful. Bone tumors often require surgery for accurate diagnosis. Surgical excision has been used in majority of cases. Other treatment modalities include radiotherapy and embolization. Recently chemoembolization and microwave ablation in combination have been used with success.<ref name="pmid25295139">{{cite journal |vauthors=Barber E, Domes T |title=Painful erections secondary to rare epithelioid hemangioma of the penis |journal=Can Urol Assoc J |volume=8 |issue=9-10 |pages=E647–9 |date=September 2014 |pmid=25295139 |pmc=4164556 |doi=10.5489/cuaj.1833 |url=}}</ref><ref name="pmid28159479">{{cite journal |vauthors=Tsikopoulos K, Perdikakis E, Georgiannos D, Bisbinas I |title=Epithelioid hemangioma of the scapula treated with chemoembolization and microwave ablation: Α case report |journal=Acta Orthop Traumatol Turc |volume=52 |issue=2 |pages=157–161 |date=March 2018 |pmid=28159479 |pmc=6136344 |doi=10.1016/j.aott.2017.01.003 |url=}}</ref><ref name="pmid26054166">{{cite journal |vauthors=Gérard V, Tomasella M, Kurth W, Brands G, Lognard M |title=[Epithelioid hemangioma, a rare bone tumor] |language=French |journal=Rev Med Liege |volume=70 |issue=4 |pages=169–71 |date=April 2015 |pmid=26054166 |doi= |url=}}</ref>
====Pyogenic granuloma====
* Also called as lobular capillary hemangioma, this common vascular lesion typically manifests as single, localized nodules on gingiva with sessile base but large lesions often present as lobulated or pediculated . Majority of the lesions are <2 cm in diameter and color of the lesion depending on vascularity varies from pink to purple. It can found anywhere on skin and mucous membranes such as lips, tongues, palate, and on atypical locations such asperiungual or gastrointestinal tract but gingiva is the typical location. Majority of the patients present with profuse bleeding. Others complain of painless mass, swelling, obstructive or interference related symptoms.<ref name="pmid27382492">{{cite journal |vauthors=Marla V, Shrestha A, Goel K, Shrestha S |title=The Histopathological Spectrum of Pyogenic Granuloma: A Case Series |journal=Case Rep Dent |volume=2016 |issue= |pages=1323798 |date=2016 |pmid=27382492 |pmc=4921146 |doi=10.1155/2016/1323798 |url=}}</ref><ref name="pmid24859551">{{cite journal |vauthors=Thada SR, Pai KM, Agarwal P |title=A huge oral pyogenic granuloma with extensive alveolar bone loss and 'sun-ray' appearance mimicking a malignant tumour |journal=BMJ Case Rep |volume=2014 |issue= |pages= |date=May 2014 |pmid=24859551 |pmc=4039851 |doi=10.1136/bcr-2013-202367 |url=}}</ref><ref name="pmid28785323">{{cite journal |vauthors=Wollina U, Langner D, França K, Gianfaldoni S, Lotti T, Tchernev G |title=Pyogenic Granuloma - A Common Benign Vascular Tumor with Variable Clinical Presentation: New Findings and Treatment Options |journal=Open Access Maced J Med Sci |volume=5 |issue=4 |pages=423–426 |date=July 2017 |pmid=28785323 |pmc=5535648 |doi=10.3889/oamjms.2017.111 |url=}}</ref><ref name="pmid22434943">{{cite journal |vauthors=Kamal R, Dahiya P, Puri A |title=Oral pyogenic granuloma: Various concepts of etiopathogenesis |journal=J Oral Maxillofac Pathol |volume=16 |issue=1 |pages=79–82 |date=January 2012 |pmid=22434943 |pmc=3303528 |doi=10.4103/0973-029X.92978 |url=}}</ref>
* Trauma or chronic irritation have been cited as the most common causes but it can arise due to multiple other factors such as medications, viral infections such as herpes virus type 1, Orf virus and/or human papilloma virus type 2, and BRAF mutations or use of BRAF inhibitors that can cause multiple, disseminated lesions. Medications that have been implicated in development of this lesion include oral contraceptives, retinoids, gefitinib, cabecitabine, and afatinib, BRAF inhibitors such as vemurafenib or encorafenib, and rituximab. Mutations in BRAF/RAS/GNA14 have all been associated with pyogenic granuloma, BRAF c.1799T>A has been recently described as one of the major mutations associated.<ref name="pmid24859551">{{cite journal |vauthors=Thada SR, Pai KM, Agarwal P |title=A huge oral pyogenic granuloma with extensive alveolar bone loss and 'sun-ray' appearance mimicking a malignant tumour |journal=BMJ Case Rep |volume=2014 |issue= |pages= |date=May 2014 |pmid=24859551 |pmc=4039851 |doi=10.1136/bcr-2013-202367 |url=}}</ref><ref name="pmid28785323">{{cite journal |vauthors=Wollina U, Langner D, França K, Gianfaldoni S, Lotti T, Tchernev G |title=Pyogenic Granuloma - A Common Benign Vascular Tumor with Variable Clinical Presentation: New Findings and Treatment Options |journal=Open Access Maced J Med Sci |volume=5 |issue=4 |pages=423–426 |date=July 2017 |pmid=28785323 |pmc=5535648 |doi=10.3889/oamjms.2017.111 |url=}}</ref><ref name="pmid22434943">{{cite journal |vauthors=Kamal R, Dahiya P, Puri A |title=Oral pyogenic granuloma: Various concepts of etiopathogenesis |journal=J Oral Maxillofac Pathol |volume=16 |issue=1 |pages=79–82 |date=January 2012 |pmid=22434943 |pmc=3303528 |doi=10.4103/0973-029X.92978 |url=}}</ref><ref name="pmid27885661">{{cite journal |vauthors=Simmons BJ, Chen L, Hu S |title=Pyogenic granuloma association with isotretinoin treatment for acne |journal=Australas. J. Dermatol. |volume=57 |issue=4 |pages=e144–e145 |date=November 2016 |pmid=27885661 |doi=10.1111/ajd.12418 |url=}}</ref><ref name="pmid26603180">{{cite journal |vauthors=Inoue A, Sawada Y, Nishio D, Nakamura M |title=Pyogenic granuloma caused by afatinib: Case report and review of the literature |journal=Australas. J. Dermatol. |volume=58 |issue=1 |pages=61–62 |date=February 2017 |pmid=26603180 |doi=10.1111/ajd.12423 |url=}}</ref><ref name="pmid27116335">{{cite journal |vauthors=Henning B, Stieger P, Kamarachev J, Dummer R, Goldinger SM |title=Pyogenic granuloma in patients treated with selective BRAF inhibitors: another manifestation of paradoxical pathway activation |journal=Melanoma Res. |volume=26 |issue=3 |pages=304–7 |date=June 2016 |pmid=27116335 |doi=10.1097/CMR.0000000000000248 |url=}}</ref><ref name="pmid26802240">{{cite journal |vauthors=Groesser L, Peterhof E, Evert M, Landthaler M, Berneburg M, Hafner C |title=BRAF and RAS Mutations in Sporadic and Secondary Pyogenic Granuloma |journal=J. Invest. Dermatol. |volume=136 |issue=2 |pages=481–6 |date=February 2016 |pmid=26802240 |doi=10.1038/JID.2015.376 |url=}}</ref>
* The diagnosis is made by clinical features and then confirmed by histopatholgical features. Current standard of care is surgical excision. Other treatment modalities include curettage, electrocautery, radiosurgery, cryosurgery, sclerotherapy, or laser treatment. Topical or oral beta-blockers timolol or propranolol and topical imiquimod have also been successful.<ref name="pmid24859551">{{cite journal |vauthors=Thada SR, Pai KM, Agarwal P |title=A huge oral pyogenic granuloma with extensive alveolar bone loss and 'sun-ray' appearance mimicking a malignant tumour |journal=BMJ Case Rep |volume=2014 |issue= |pages= |date=May 2014 |pmid=24859551 |pmc=4039851 |doi=10.1136/bcr-2013-202367 |url=}}</ref><ref name="pmid28785323">{{cite journal |vauthors=Wollina U, Langner D, França K, Gianfaldoni S, Lotti T, Tchernev G |title=Pyogenic Granuloma - A Common Benign Vascular Tumor with Variable Clinical Presentation: New Findings and Treatment Options |journal=Open Access Maced J Med Sci |volume=5 |issue=4 |pages=423–426 |date=July 2017 |pmid=28785323 |pmc=5535648 |doi=10.3889/oamjms.2017.111 |url=}}</ref><ref name="pmid17478135">{{cite journal |vauthors=Giblin AV, Clover AJ, Athanassopoulos A, Budny PG |title=Pyogenic granuloma - the quest for optimum treatment: audit of treatment of 408 cases |journal=J Plast Reconstr Aesthet Surg |volume=60 |issue=9 |pages=1030–5 |date=2007 |pmid=17478135 |doi=10.1016/j.bjps.2006.10.018 |url=}}</ref><ref name="pmid27874157">{{cite journal |vauthors=Al-Mohaya MA, Al-Malik AM |title=Excision of oral pyogenic granuloma in a diabetic patient with 940nm diode laser |journal=Saudi Med J |volume=37 |issue=12 |pages=1395–1400 |date=December 2016 |pmid=27874157 |pmc=5303780 |doi=10.15537/smj.2016.12.15941 |url=}}</ref><ref name="pmid24836966">{{cite journal |vauthors=Malik M, Murphy R |title=A pyogenic granuloma treated with topical timolol |journal=Br. J. Dermatol. |volume=171 |issue=6 |pages=1537–8 |date=December 2014 |pmid=24836966 |doi=10.1111/bjd.13116 |url=}}</ref><ref name="pmid24656264">{{cite journal |vauthors=Millsop JW, Trinh N, Winterfield L, Berrios R, Hutchens KA, Tung R |title=Resolution of recalcitrant pyogenic granuloma with laser, corticosteroid, and timolol therapy |journal=Dermatol. Online J. |volume=20 |issue=3 |pages= |date=March 2014 |pmid=24656264 |doi= |url=}}</ref>
====Hobnail hemangioma====
* Benign tumor that typically presents as solitary growth with often, but not always, tagetoid appearance of a central papule and peripheral brown ring that may or may not disappear over time. Characteristic histopathological feature include plump endothelial cells in superficial dermis that line ectatic and irregular vessels, and project into lumina like hobnails. Deeper dermis shows vessels dissecting collagen fibers. Majority of the lesions are fund on trunk and extremities with head and neck, and oral cavity as uncommon locations. Patient may present with pain, or an asymptomatic growing lesion.<ref name="pmid23808782">{{cite journal |vauthors=Hiremath SK, Charantimath S, Byakodi S, Bijjal S, Byakodi R, Sapra G |title=Oral hobnail hemangioma: a case report |journal=Arch Iran Med |volume=16 |issue=7 |pages=428–30 |date=July 2013 |pmid=23808782 |doi=013167/AIM.0013 |url=}}</ref><ref name="pmid22148030">{{cite journal |vauthors=Yoon SY, Kwon HH, Jeon HC, Lee JH, Cho S |title=Congenital and multiple hobnail hemangiomas |journal=Ann Dermatol |volume=23 |issue=4 |pages=539–43 |date=November 2011 |pmid=22148030 |pmc=3229956 |doi=10.5021/ad.2011.23.4.539 |url=}}</ref><ref name="pmid26156669">{{cite journal |vauthors=Takayama R, Ueno T, Futagami A, Ansai S, Fukumoto T, Saeki H |title=Hobnail Hemangioma: A Case Report |journal=J Nippon Med Sch |volume=82 |issue=3 |pages=151–5 |date=2015 |pmid=26156669 |doi=10.1272/jnms.82.151 |url=}}</ref>
* Etiology is not well understood but trauma may play a key role in pathogenesis. Some studies have found congenital etiology in some lesions.<ref name="pmid23808782">{{cite journal |vauthors=Hiremath SK, Charantimath S, Byakodi S, Bijjal S, Byakodi R, Sapra G |title=Oral hobnail hemangioma: a case report |journal=Arch Iran Med |volume=16 |issue=7 |pages=428–30 |date=July 2013 |pmid=23808782 |doi=013167/AIM.0013 |url=}}</ref><ref name="pmid22148030">{{cite journal |vauthors=Yoon SY, Kwon HH, Jeon HC, Lee JH, Cho S |title=Congenital and multiple hobnail hemangiomas |journal=Ann Dermatol |volume=23 |issue=4 |pages=539–43 |date=November 2011 |pmid=22148030 |pmc=3229956 |doi=10.5021/ad.2011.23.4.539 |url=}}</ref><ref name="pmid7829718">{{cite journal |vauthors=Morganroth GS, Tigelaar RE, Longley BJ, Luck LE, Leffell DJ |title=Targetoid hemangioma associated with pregnancy and the menstrual cycle |journal=J. Am. Acad. Dermatol. |volume=32 |issue=2 Pt 1 |pages=282–4 |date=February 1995 |pmid=7829718 |doi= |url=}}</ref><ref name="pmid11391103">{{cite journal |vauthors=Christenson LJ, Stone MS |title=Trauma-induced simulator of targetoid hemosiderotic hemangioma |journal=Am J Dermatopathol |volume=23 |issue=3 |pages=221–3 |date=June 2001 |pmid=11391103 |doi= |url=}}</ref>
* Diagnosis is based on clinical features and histopathological studies. Treatment is usually by excision. Other modalities of treatment include intermittent triamcinolone intralesional injections and pulsed dye laser treatment.<ref name="pmid23808782">{{cite journal |vauthors=Hiremath SK, Charantimath S, Byakodi S, Bijjal S, Byakodi R, Sapra G |title=Oral hobnail hemangioma: a case report |journal=Arch Iran Med |volume=16 |issue=7 |pages=428–30 |date=July 2013 |pmid=23808782 |doi=013167/AIM.0013 |url=}}</ref><ref name="pmid22148030">{{cite journal |vauthors=Yoon SY, Kwon HH, Jeon HC, Lee JH, Cho S |title=Congenital and multiple hobnail hemangiomas |journal=Ann Dermatol |volume=23 |issue=4 |pages=539–43 |date=November 2011 |pmid=22148030 |pmc=3229956 |doi=10.5021/ad.2011.23.4.539 |url=}}</ref>
====Microvenular hemangioma====
* Rare lesion that most often manifests as single asymptomatic nodule, plaque or papule with color varying from red to bluish-red. Majority of the lesions are located on trunk and limbs. Histologically, the tumor consists of irregular and branching venous structures with inconspicuous lumina. Endothelial cells display absence of atypia and mitotic figures. Some lesions may be painful and/or tender.<ref name="pmid29445567">{{cite journal |vauthors=Mansur AT, Demirci GT, Ozbal Koc EA, Yildiz S |title=An unusual lesion on the nose: microvenular hemangioma |journal=Dermatol Pract Concept |volume=8 |issue=1 |pages=7–11 |date=January 2018 |pmid=29445567 |pmc=5808364 |doi=10.5826/dpc.0801a02 |url=}}</ref><ref name="pmid9418757">{{cite journal |vauthors=Requena L, Sangueza OP |title=Cutaneous vascular proliferation. Part II. Hyperplasias and benign neoplasms |journal=J. Am. Acad. Dermatol. |volume=37 |issue=6 |pages=887–919; quiz 920–2 |date=December 1997 |pmid=9418757 |doi= |url=}}</ref><ref name="pmid25138781">{{cite journal |vauthors=Koch PS, Goerdt S, Peitsch WK |title=Solitary red-purple plaque on the chest of a 7-year-old boy: a quiz. Microvenular haemangioma |journal=Acta Derm. Venereol. |volume=95 |issue=3 |pages=378–82 |date=March 2015 |pmid=25138781 |doi=10.2340/00015555-1953 |url=}}</ref><ref name="pmid12592086">{{cite journal |vauthors=Kim YC, Park HJ, Cinn YW |title=Microvenular hemangioma |journal=Dermatology (Basel) |volume=206 |issue=2 |pages=161–4 |date=2003 |pmid=12592086 |doi=10.1159/000068453 |url=}}</ref>
* Etiology and pathogenesis have not been well-understood but a recent study may associate progesterone with microvenular hemangioma.<ref name="pmid30019384">{{cite journal |vauthors=Juan YC, Chen CJ, Hsiao CH, Chiu TM |title=A microvenular hemangioma with a rare expression of progesterone receptor immunocreativity and a review of the literature |journal=J. Cutan. Pathol. |volume= |issue= |pages= |date=July 2018 |pmid=30019384 |doi=10.1111/cup.13322 |url=}}</ref>
* Diagnosis requires biopsy because of rarity of this tumor. Treatment is through surgical excision.<ref name="pmid29445567">{{cite journal |vauthors=Mansur AT, Demirci GT, Ozbal Koc EA, Yildiz S |title=An unusual lesion on the nose: microvenular hemangioma |journal=Dermatol Pract Concept |volume=8 |issue=1 |pages=7–11 |date=January 2018 |pmid=29445567 |pmc=5808364 |doi=10.5826/dpc.0801a02 |url=}}</ref><ref name="pmid9418757">{{cite journal |vauthors=Requena L, Sangueza OP |title=Cutaneous vascular proliferation. Part II. Hyperplasias and benign neoplasms |journal=J. Am. Acad. Dermatol. |volume=37 |issue=6 |pages=887–919; quiz 920–2 |date=December 1997 |pmid=9418757 |doi= |url=}}</ref><ref name="pmid25138781">{{cite journal |vauthors=Koch PS, Goerdt S, Peitsch WK |title=Solitary red-purple plaque on the chest of a 7-year-old boy: a quiz. Microvenular haemangioma |journal=Acta Derm. Venereol. |volume=95 |issue=3 |pages=378–82 |date=March 2015 |pmid=25138781 |doi=10.2340/00015555-1953 |url=}}</ref><ref name="pmid12592086">{{cite journal |vauthors=Kim YC, Park HJ, Cinn YW |title=Microvenular hemangioma |journal=Dermatology (Basel) |volume=206 |issue=2 |pages=161–4 |date=2003 |pmid=12592086 |doi=10.1159/000068453 |url=}}</ref>
====Anastomosing hemangioma====
* Rare tumor characterized by presence of anastomosing capillary-sized vessels with irregular fenestration, lined by endothelial cells with absence of atypia and occasional hobnailing. Majority of the lesions are found in kidneys and genitourinary tract with some being located in liver, adrenal glands and gastrointestinal tract. These tumors are typically incidental findings on radiology but some may present with back and/or flank pain with/without radiation to lower limbs.<ref name="pmid26893881">{{cite journal |vauthors=Jin LU, Liu J, Li Y, Sun S, Mao X, Yang S, Lai Y |title=Anastomosing hemangioma: The first case report in the bladder |journal=Mol Clin Oncol |volume=4 |issue=2 |pages=310–312 |date=February 2016 |pmid=26893881 |pmc=4734195 |doi=10.3892/mco.2015.699 |url=}}</ref><ref name="pmid22840861">{{cite journal |vauthors=Ross M, Polcari A, Picken M, Sankary H, Milner J |title=Anastomosing hemangioma arising from the adrenal gland |journal=Urology |volume=80 |issue=3 |pages=e27–8 |date=September 2012 |pmid=22840861 |doi=10.1016/j.urology.2012.05.032 |url=}}</ref><ref name="pmid23887160">{{cite journal |vauthors=Lin J, Bigge J, Ulbright TM, Montgomery E |title=Anastomosing hemangioma of the liver and gastrointestinal tract: an unusual variant histologically mimicking angiosarcoma |journal=Am. J. Surg. Pathol. |volume=37 |issue=11 |pages=1761–5 |date=November 2013 |pmid=23887160 |doi=10.1097/PAS.0b013e3182967e6c |url=}}</ref><ref name="pmid29962849">{{cite journal |vauthors=Cheon PM, Rebello R, Naqvi A, Popovic S, Bonert M, Kapoor A |title=Anastomosing hemangioma of the kidney: radiologic and pathologic distinctions of a kidney cancer mimic |journal=Curr Oncol |volume=25 |issue=3 |pages=e220–e223 |date=June 2018 |pmid=29962849 |pmc=6023565 |doi=10.3747/co.25.3927 |url=}}</ref><ref name="pmid28118845">{{cite journal |vauthors=Perdiki M, Datseri G, Liapis G, Chondros N, Anastasiou I, Tzardi M, Delladetsima JK, Drakos E |title=Anastomosing hemangioma: report of two renal cases and analysis of the literature |journal=Diagn Pathol |volume=12 |issue=1 |pages=14 |date=January 2017 |pmid=28118845 |pmc=5260082 |doi=10.1186/s13000-017-0597-4 |url=}}</ref>
* Etiology is not well-known but a recent study suggests muations in GNAQ genes that encodes members of G protein family.<ref name="pmid28084343">{{cite journal |vauthors=Bean GR, Joseph NM, Gill RM, Folpe AL, Horvai AE, Umetsu SE |title=Recurrent GNAQ mutations in anastomosing hemangiomas |journal=Mod. Pathol. |volume=30 |issue=5 |pages=722–727 |date=May 2017 |pmid=28084343 |doi=10.1038/modpathol.2016.234 |url=}}</ref>
* The diagnosis is very challenging because radiological findings of anastomosing hemangioma are similar to that of malignant tumors such as renal cell carcinoma, hepatocellular carcinoma and low grade angiosarcoma. So excision and biopsy are often suggested. There is no current guidelines for treatment but excision and local ablation are the options.<ref name="pmid26893881">{{cite journal |vauthors=Jin LU, Liu J, Li Y, Sun S, Mao X, Yang S, Lai Y |title=Anastomosing hemangioma: The first case report in the bladder |journal=Mol Clin Oncol |volume=4 |issue=2 |pages=310–312 |date=February 2016 |pmid=26893881 |pmc=4734195 |doi=10.3892/mco.2015.699 |url=}}</ref><ref name="pmid29962849">{{cite journal |vauthors=Cheon PM, Rebello R, Naqvi A, Popovic S, Bonert M, Kapoor A |title=Anastomosing hemangioma of the kidney: radiologic and pathologic distinctions of a kidney cancer mimic |journal=Curr Oncol |volume=25 |issue=3 |pages=e220–e223 |date=June 2018 |pmid=29962849 |pmc=6023565 |doi=10.3747/co.25.3927 |url=}}</ref><ref name="pmid23887160">{{cite journal |vauthors=Lin J, Bigge J, Ulbright TM, Montgomery E |title=Anastomosing hemangioma of the liver and gastrointestinal tract: an unusual variant histologically mimicking angiosarcoma |journal=Am. J. Surg. Pathol. |volume=37 |issue=11 |pages=1761–5 |date=November 2013 |pmid=23887160 |doi=10.1097/PAS.0b013e3182967e6c |url=}}</ref><ref name="pmid29279869">{{cite journal |vauthors=Abboudi H, Tschobotko B, Carr C, DasGupta R |title=Bilateral Renal Anastomosing Hemangiomas: A Tale of Two Kidneys |journal=J Endourol Case Rep |volume=3 |issue=1 |pages=176–178 |date=2017 |pmid=29279869 |pmc=5734139 |doi=10.1089/cren.2017.0018 |url=}}</ref><ref name="pmid28808574">{{cite journal |vauthors=Peng X, Li J, Liang Z |title=Anastomosing haemangioma of liver: A case report |journal=Mol Clin Oncol |volume=7 |issue=3 |pages=507–509 |date=September 2017 |pmid=28808574 |pmc=5543263 |doi=10.3892/mco.2017.1341 |url=}}</ref>
====Glomeruloid hemangioma====
* Characterized by red blood cells filled clumps of capillaries inside dilated vascular spaces. These collections of capillaries, lined by swollen endothelial cells, resemble renal glomeruli and stain positive for periodic acid-Schiff (PAS)-positive, diastase-resistant eosinophilic globules. Clinical presentation varies and are not discernible from other cutaneous lesions. Majority of the lesions manifest as multiple, asymptomatic pauples or nodules..<ref name="pmid23716835">{{cite journal |vauthors=Gupta J, Kandhari R, Ramesh V, Singh A |title=Glomeruloid hemangioma in normal individuals |journal=Indian J Dermatol |volume=58 |issue=2 |pages=160 |date=March 2013 |pmid=23716835 |pmc=3657245 |doi=10.4103/0019-5154.108088 |url=}}</ref><ref name="pmid27279314">{{cite journal |vauthors=Gupta V, Rai A, Mridha AR, Sharma VK |title=Multiple glomeruloid hemangiomas without POEMS syndrome |journal=Indian J Dermatol Venereol Leprol |volume=82 |issue=4 |pages=442–4 |date=2016 |pmid=27279314 |doi=10.4103/0378-6323.181461 |url=}}</ref>
* Glomeruloid hemangioma is associated with POEMS syndrome in majority of the cases and rarely with Castleman's disease. Very few isolated cases of glomeruloid hemangioma have been reported.<ref name="pmid23716835">{{cite journal |vauthors=Gupta J, Kandhari R, Ramesh V, Singh A |title=Glomeruloid hemangioma in normal individuals |journal=Indian J Dermatol |volume=58 |issue=2 |pages=160 |date=March 2013 |pmid=23716835 |pmc=3657245 |doi=10.4103/0019-5154.108088 |url=}}</ref><ref name="pmid27279314">{{cite journal |vauthors=Gupta V, Rai A, Mridha AR, Sharma VK |title=Multiple glomeruloid hemangiomas without POEMS syndrome |journal=Indian J Dermatol Venereol Leprol |volume=82 |issue=4 |pages=442–4 |date=2016 |pmid=27279314 |doi=10.4103/0378-6323.181461 |url=}}</ref>
** POEMS stands for peripheral neuropathy (P), organomegaly (O), endocrinopathy (E) monoclonal plasma-cells proliferative disorder (M) and skin changes (S) although diagnosis does not require presence of all of these symptoms. Other manifestations of POEMS syndrome may include sclerotic bone lesions, papilledema, edema, ascites, effusions, pulmonary hypertension, Castleman’ disease (CD), thrombocytosis and erythrocytosis, and increased serum VEGF.<ref name="pmid28894560">{{cite journal |vauthors=Nozza A |title=POEMS SYNDROME: an Update |journal=Mediterr J Hematol Infect Dis |volume=9 |issue=1 |pages=e2017051 |date=2017 |pmid=28894560 |pmc=5584767 |doi=10.4084/MJHID.2017.051 |url=}}</ref>
** Castleman’s disease is characterized as lymphoproliferative disorder with inflammatory response involving multiple systems. Clinical presentation ranges from asymptomatic lymphadenopathy to severe systemic manifestations such as weight loss, fever and organomegaly.<ref name="pmid21499466">{{cite journal |vauthors=Kahn F, Fagerström A, Segelmark M, Bakoush O |title=Irreversible Kidney Damage due to Multicentric Castleman's Disease |journal=Libyan J Med |volume=3 |issue=2 |pages=101–3 |date=June 2008 |pmid=21499466 |pmc=3074288 |doi=10.4176/080108 |url=}}</ref>
* Etiology is not well-understood but some theories suggest role of vascular endothelial growth factor (VEGF), increased estrogen levels, human herpesvirus-8 and increased cytokines in its pathogenesis.<ref name="pmid23716835">{{cite journal |vauthors=Gupta J, Kandhari R, Ramesh V, Singh A |title=Glomeruloid hemangioma in normal individuals |journal=Indian J Dermatol |volume=58 |issue=2 |pages=160 |date=March 2013 |pmid=23716835 |pmc=3657245 |doi=10.4103/0019-5154.108088 |url=}}</ref><ref name="pmid27279314">{{cite journal |vauthors=Gupta V, Rai A, Mridha AR, Sharma VK |title=Multiple glomeruloid hemangiomas without POEMS syndrome |journal=Indian J Dermatol Venereol Leprol |volume=82 |issue=4 |pages=442–4 |date=2016 |pmid=27279314 |doi=10.4103/0378-6323.181461 |url=}}</ref>
* Diagnosis relies on characteristic histology. Patients who present with glomeruloid hemangioma should undergo evaluation for POEMS syndrome and should be kept under follow-up because these lesions can precede full-blown POEMS syndrome in some cases.<ref name="pmid23716835">{{cite journal |vauthors=Gupta J, Kandhari R, Ramesh V, Singh A |title=Glomeruloid hemangioma in normal individuals |journal=Indian J Dermatol |volume=58 |issue=2 |pages=160 |date=March 2013 |pmid=23716835 |pmc=3657245 |doi=10.4103/0019-5154.108088 |url=}}</ref><ref name="pmid27279314">{{cite journal |vauthors=Gupta V, Rai A, Mridha AR, Sharma VK |title=Multiple glomeruloid hemangiomas without POEMS syndrome |journal=Indian J Dermatol Venereol Leprol |volume=82 |issue=4 |pages=442–4 |date=2016 |pmid=27279314 |doi=10.4103/0378-6323.181461 |url=}}</ref>
====Papillary hemangioma====
* Rare tumor characterized by presence of papillary growths within dilated vascular channels, that contain cores of pericytes around normal capillaries. The tumor typically manifests as solitary papules in head and neck region, without any systemic manifestations.<ref name="pmid18805868">{{cite journal |vauthors=Suurmeijer AJ |title=Papillary hemangiomas and glomeruloid hemangiomas are distinct clinicopathological entities |journal=Int. J. Surg. Pathol. |volume=18 |issue=1 |pages=48–54 |date=February 2010 |pmid=18805868 |doi=10.1177/1066896908323504 |url=}}</ref><ref name="pmid17927585">{{cite journal |vauthors=Suurmeijer AJ, Fletcher CD |title=Papillary haemangioma. A distinctive cutaneous haemangioma of the head and neck area containing eosinophilic hyaline globules |journal=Histopathology |volume=51 |issue=5 |pages=638–48 |date=November 2007 |pmid=17927585 |doi=10.1111/j.1365-2559.2007.02847.x |url=}}</ref>
* Diagnosis may require biopsy. Treatment options include excision and photodynamic therapy.<ref name="pmid12093647">{{cite journal |vauthors=Schmidt-Erfurth UM, Kusserow C, Barbazetto IA, Laqua H |title=Benefits and complications of photodynamic therapy of papillary capillary hemangiomas |journal=Ophthalmology |volume=109 |issue=7 |pages=1256–66 |date=July 2002 |pmid=12093647 |doi= |url=}}</ref>
====Intravascular papillary endothelial hyperplasia====
* Also called as Masson's tumor, this benign lesions is characterized by presence of intravascular papillary structures that are enveloped by proliferating endothelial cells. It is considered to be a reactive lesion associated with an organizing thrombus. Clinically it manifests as solitary painless mass in head-neck and the extremities especially the hand, that may grow rapidly in size and become painful and/or tender. Some lesions have been found intra-abdominallly such as in the liver that can bleed and present with anemia.<ref name="pmid24125024">{{cite journal |vauthors=Akdur NC, Donmez M, Gozel S, Ustun H, Hucumenoglu S |title=Intravascular papillary endothelial hyperplasia: histomorphological and immunohistochemical features |journal=Diagn Pathol |volume=8 |issue= |pages=167 |date=October 2013 |pmid=24125024 |pmc=4016006 |doi=10.1186/1746-1596-8-167 |url=}}</ref><ref name="pmid24294378">{{cite journal |vauthors=Kim S, Jun JH, Kim J, Kim DW, Jang YH, Lee WJ, Chung HY, Lee SJ |title=HIF-1α and VEGF expression correlates with thrombus remodeling in cases of intravascular papillary endothelial hyperplasia |journal=Int J Clin Exp Pathol |volume=6 |issue=12 |pages=2912–8 |date=2013 |pmid=24294378 |pmc=3843272 |doi= |url=}}</ref><ref name="pmid24891960">{{cite journal |vauthors=Guledgud MV, Patil K, Saikrishna D, Madhavan A, Yelamali T |title=Intravascular papillary endothelial hyperplasia: diagnostic sequence and literature review of an orofacial lesion |journal=Case Rep Dent |volume=2014 |issue= |pages=934593 |date=2014 |pmid=24891960 |pmc=4033548 |doi=10.1155/2014/934593 |url=}}</ref><ref name="pmid15082910">{{cite journal |vauthors=Hong SG, Cho HM, Chin HM, Park IY, Yoo JY, Hwang SS, Kim JG, Park WB, Chun CS |title=Intravascular papillary endothelial hyperplasia (Masson's hemangioma) of the liver: a new hepatic lesion |journal=J. Korean Med. Sci. |volume=19 |issue=2 |pages=305–8 |date=April 2004 |pmid=15082910 |pmc=2822318 |doi=10.3346/jkms.2004.19.2.305 |url=}}</ref>
* This lesions appears to be associated with vascular trauma, and thrombus that may lead to chronic irritation and increased levels of fibroblast growth factor (FGF), hypoxia-inducible factor-1 (HIF-1α), and vascular endothelial growth factor (VEGF) stimulating endothelial cells proliferation.<ref name="pmid24294378"></ref><ref name="pmid24891960"></ref><ref name="pmid26225335">{{cite journal |vauthors=Mahapatra QS, Sahai K, Malik A, Mani NS |title=Intravascular papillary endothelial hyperplasia: An unusual histopathological entity |journal=Indian Dermatol Online J |volume=6 |issue=4 |pages=277–9 |date=2015 |pmid=26225335 |pmc=4513410 |doi=10.4103/2229-5178.160269 |url=}}</ref>
* Histopathological studies are generally required for diagnosis and may also require immunohistochemical confirmation. Treatment is surgery with uncommon recurrence.<ref name="pmid26225335"></ref><ref name="pmid21103239">{{cite journal |vauthors=Meadows MC, Sun X, Dardik M, Tarantino DR, Chamberlain RS |title=Intraabdominal Intravascular Papillary Endothelial Hyperplasia (Masson's Tumor): A Rare and Novel Cause of Gastrointestinal Bleeding |journal=Case Rep Gastroenterol |volume=4 |issue=1 |pages=124–132 |date=March 2010 |pmid=21103239 |pmc=2988909 |doi=10.1159/000294148 |url=}}</ref>
====Cutaneous epithelioid angiomatous nodule====
* This recently diagnosed lesion is characterized histologically as proliferating, swollen epithelioid cells and thin walled vascular channels lined by swollen endothelial cells that resemble the epithelioid cells. These cells typically contain pale pink cytoplasm, intracytoplasmic vacuoles, and vesicular nuclei. Clinically these lesions manifest as solitary nodules or papules that may grow rapidly. Rarely multiple lesions have also been reported.<ref name="pmid27981217">{{cite journal |vauthors=Blackwood L, Chapman I, Lyon M, Hernandez C |title=Multifocal eruptive cutaneous epithelioid angiomatous nodules |journal=JAAD Case Rep |volume=2 |issue=6 |pages=454–456 |date=November 2016 |pmid=27981217 |pmc=5148776 |doi=10.1016/j.jdcr.2016.09.013 |url=}}</ref><ref name="pmid30103782">{{cite journal |vauthors=Chetty R, Kamil ZS, Wang A, Al Habeeb A, Ghazarian D |title=Cutaneous epithelioid angiomatous nodule: a report of a series including a case with moderate cytologic atypia and immunosuppression |journal=Diagn Pathol |volume=13 |issue=1 |pages=50 |date=August 2018 |pmid=30103782 |pmc=6090800 |doi=10.1186/s13000-018-0729-5 |url=}}</ref><ref name="pmid23551998">{{cite journal |vauthors=Dastgheib L, Aslani FS, Sepaskhah M, Saki N, Motevalli D |title=A young woman with multiple cutaneous epithelioid angiomatous nodules (CEAN) on her forearm: a case report and follow-up of therapeutic intervention |journal=Dermatol. Online J. |volume=19 |issue=3 |pages=1 |date=March 2013 |pmid=23551998 |doi= |url=}}</ref>
* Thought to be a reactive process but no trigger has been identified yet. Immunosuppression has been proposed to be associated with this benign vascular proliferation.<ref name="pmid27981217"></ref><ref name="pmid30103782"></ref>
* Diagnosis requires careful clinical and histopathological evaluation. Surgery has been the treatment used in majority of the lesions although cryotherapy has also been used. Steroids are found to be ineffective.<ref name="pmid27981217"></ref><ref name="pmid23551998">{{cite journal |vauthors=Dastgheib L, Aslani FS, Sepaskhah M, Saki N, Motevalli D |title=A young woman with multiple cutaneous epithelioid angiomatous nodules (CEAN) on her forearm: a case report and follow-up of therapeutic intervention |journal=Dermatol. Online J. |volume=19 |issue=3 |pages=1 |date=March 2013 |pmid=23551998 |doi= |url=}}</ref>
====Acquired elastotic hemangioma====
* First described in 2002, this rare lesion typically manifests as solitary, asymptomatic, red to purple, patches and plaques on areas damaged by sun exposure such as hands and forearms. Some patients may complain of pain or growth of the lesion. Histologically its exhibits solar elastosis and band like proliferation of capillaries in superficial dermis. These proliferating channels are arranged parallel to epidermis and endothelial cells typically display absence of atypia but may show hobnail pattern.<ref name="pmid27867745">{{cite journal |vauthors=Hicks T, Katz I |title=First description of the dermatoscopic features of acquired elastotic hemangioma-a case report |journal=Dermatol Pract Concept |volume=6 |issue=4 |pages=35–37 |date=October 2016 |pmid=27867745 |pmc=5108644 |doi=10.5826/dpc.0604a08 |url=}}</ref><ref name="pmid29507844">{{cite journal |vauthors=Cohen PR, Hinds BR |title=Acquired Elastotic Hemangioma: Case Series and Comprehensive Literature Review |journal=Cureus |volume=9 |issue=12 |pages=e1994 |date=December 2017 |pmid=29507844 |pmc=5832390 |doi=10.7759/cureus.1994 |url=}}</ref>
* This lesion has slight preference for females. Damage due to sun exposure such as free radical production are thought to be associated with pathogenesis. Some case reports showed use of progesterone associated with appearance of lesions in perimenopausal women.<ref name="pmid27867745"></ref><ref name="pmid29507844"></ref><ref name="pmid12196746">{{cite journal |vauthors=Requena L, Kutzner H, Mentzel T |title=Acquired elastotic hemangioma: A clinicopathologic variant of hemangioma |journal=J. Am. Acad. Dermatol. |volume=47 |issue=3 |pages=371–6 |date=September 2002 |pmid=12196746 |doi= |url=}}</ref><ref name="pmid20950363">{{cite journal |vauthors=Tong PL, Beer TW |title=Acquired elastotic hemangioma: ten cases with immunohistochemistry refuting a lymphatic origin in most lesions |journal=J. Cutan. Pathol. |volume=37 |issue=12 |pages=1259–60 |date=December 2010 |pmid=20950363 |doi=10.1111/j.1600-0560.2010.01610.x |url=}}</ref>
* Diagnosis requires biopsy to rule out basal cell carcinoma and other similar appearing lesions. Treatment options include observation, discontinuance of progesterone, laser therapy and excision. Recurrence was not observed in any of the cases observed or treated.<ref name="pmid29507844"></ref><ref name="pmid20950363"></ref><ref name="pmid27867745"></ref><ref name="pmid20950363">{{cite journal |vauthors=Tong PL, Beer TW |title=Acquired elastotic hemangioma: ten cases with immunohistochemistry refuting a lymphatic origin in most lesions |journal=J. Cutan. Pathol. |volume=37 |issue=12 |pages=1259–60 |date=December 2010 |pmid=20950363 |doi=10.1111/j.1600-0560.2010.01610.x |url=}}</ref><ref name="pmid28296791">{{cite journal |vauthors=Mendieta-Eckert M, Díaz-Ramón JL, Gardeazabal-García J |title=Response of an Acquired Elastotic Hemangioma to Vascular Laser |journal=Dermatol Surg |volume=44 |issue=1 |pages=136–137 |date=January 2018 |pmid=28296791 |doi=10.1097/DSS.0000000000001105 |url=}}</ref>
====Littoral cell hemangioma of the spleen====
* Benign tumor that originates from cells that line the red pup sinuses. Majority of the patients are asymptomatic but some present with symptoms related to splenic enlargement such as abdominal distension, hypersplenism such as persistent anemia and thrombocytopenia, and constitutional symptoms such as weight loss, fever, and fatigue. Histopathological features include anastomosing vascular channels lined by cuboidal cells, with somewhat rarer presence of papillary structures.<ref name="pmid22172167">{{cite journal |vauthors=Hu ZQ, A YJ, Sun QM, Li W, Li L |title=The splenic Littoral cell angioma in China: a case report and review |journal=World J Surg Oncol |volume=9 |issue= |pages=168 |date=December 2011 |pmid=22172167 |pmc=3271992 |doi=10.1186/1477-7819-9-168 |url=}}</ref><ref name="pmid26604237">{{cite journal |vauthors=de Ridder GG, Galeotti J, Carney J, Wang E |title=Persistent thrombocytopaenia in a young man with splenomegaly, rebound thrombocytosis after splenectomy and subsequent pulmonary embolism: splenic littoral cell angioma and associated events |journal=BMJ Case Rep |volume=2015 |issue= |pages= |date=November 2015 |pmid=26604237 |pmc=4680245 |doi=10.1136/bcr-2015-212882 |url=}}</ref><ref name="pmid18713469">{{cite journal |vauthors=Tee M, Vos P, Zetler P, Wiseman SM |title=Incidental littoral cell angioma of the spleen |journal=World J Surg Oncol |volume=6 |issue= |pages=87 |date=August 2008 |pmid=18713469 |pmc=2527567 |doi=10.1186/1477-7819-6-87 |url=}}</ref><ref name="pmid18161935">{{cite journal |vauthors=Cosme A, Tejada A, Bujanda L, Vaquero M, Elorza JL, Ojeda E, Goikoetxea U |title=Littoral-cell angioma of the spleen: a case report |journal=World J. Gastroenterol. |volume=13 |issue=48 |pages=6603–4 |date=December 2007 |pmid=18161935 |pmc=4611304 |doi= |url=}}</ref>
* Although not well-established, few case reports have demonstrated malignant histology and features in this neoplasm. This tumor can be related to visceral malignancies such as non-Hodgkin's lymphoma, tumors of the liver and brain, epithelial ovarian cancer, non-small cell lung cancer, plasmablastic B-cell lymphoma, villous lymphocyte leukaemia and neoplasm of colon, kidney and pancreas. Association with Crohn's disease and Gaucher's disease has also been described leading to hypothesis of immune dysfunction as a possible cause of littoral cell hemangioma of splen.<ref name="pmid26604237"></ref><ref name="pmid18713469"></ref><ref name="pmid18161935"></ref><ref name="pmid16971207">{{cite journal |vauthors=Harmon RL, Cerruto CA, Scheckner A |title=Littoral cell angioma: a case report and review |journal=Curr Surg |volume=63 |issue=5 |pages=345–50 |date=2006 |pmid=16971207 |doi=10.1016/j.cursur.2006.06.011 |url=}}</ref><ref name="pmid22098375">{{cite journal |vauthors=Melzer N, Barth PJ, Müller KM, Foss HD, Krug U, Schilling M, Marziniak M, Grauer OM, Wiendl H |title=Rapidly progressive B-cell dominated inflammatory neuropathy and littoral cell angioma of the spleen associated with plasmablastic B-cell lymphoma |journal=Leuk. Lymphoma |volume=53 |issue=6 |pages=1242–4 |date=June 2012 |pmid=22098375 |doi=10.3109/10428194.2011.640677 |url=}}</ref><ref name="pmid12405918">{{cite journal |vauthors=Chatelain D, Bonte H, Guillevin L, Balladur P, Flejou JF |title=Small solitary littoral cell angioma associated with splenic marginal zone lymphoma and villous lymphocyte leukaemia in a patient with hepatitis C infection |journal=Histopathology |volume=41 |issue=5 |pages=473–5 |date=November 2002 |pmid=12405918 |doi= |url=}}</ref><ref name="pmid9862523">{{cite journal |vauthors=Bisceglia M, Sickel JZ, Giangaspero F, Gomes V, Amini M, Michal M |title=Littoral cell angioma of the spleen: an additional report of four cases with emphasis on the association with visceral organ cancers |journal=Tumori |volume=84 |issue=5 |pages=595–9 |date=1998 |pmid=9862523 |doi= |url=}}</ref><ref name="pmid25705528">{{cite journal |vauthors=Johansson J, Björnsson B, Ignatova S, Sandström P, Ekstedt M |title=Littoral cell angioma in a patient with Crohn's disease |journal=Case Rep Gastrointest Med |volume=2015 |issue= |pages=474969 |date=2015 |pmid=25705528 |pmc=4326338 |doi=10.1155/2015/474969 |url=}}</ref><ref name="pmid11559940">{{cite journal |vauthors=Gupta MK, Levin M, Aguilera NS, Pastores GM |title=Littoral cell angioma of the spleen in a patient with Gaucher disease |journal=Am. J. Hematol. |volume=68 |issue=1 |pages=61–2 |date=September 2001 |pmid=11559940 |doi= |url=}}</ref><ref name="pmid7573679">{{cite journal |vauthors=Rosso R, Paulli M, Gianelli U, Boveri E, Stella G, Magrini U |title=Littoral cell angiosarcoma of the spleen. Case report with immunohistochemical and ultrastructural analysis |journal=Am. J. Surg. Pathol. |volume=19 |issue=10 |pages=1203–8 |date=October 1995 |pmid=7573679 |doi= |url=}}</ref>
* Preferred diagnostic modality is biopsy often following splenectomy. Fina needle aspiration can also be used. Imaging studies such as MRI and CT scan are inconclusive. All patients with this neoplasm should undergo evaluation for other associated neoplasms especially visceral neoplasms. Splenectomy is the treatment.<ref name="pmid22172167"></ref><ref name="pmid18713469"></ref><ref name="pmid17449783">{{cite journal |vauthors=Bhatt S, Huang J, Dogra V |title=Littoral cell angioma of the spleen |journal=AJR Am J Roentgenol |volume=188 |issue=5 |pages=1365–6 |date=May 2007 |pmid=17449783 |doi=10.2214/AJR.06.1157 |url=}}</ref><ref name="pmid25820845">{{cite journal |vauthors=Vancauwenberghe T, Snoeckx A, Vanbeckevoort D, Dymarkowski S, Vanhoenacker FM |title=Imaging of the spleen: what the clinician needs to know |journal=Singapore Med J |volume=56 |issue=3 |pages=133–44 |date=March 2015 |pmid=25820845 |pmc=4371192 |doi= |url=}}</ref><ref name="pmid28607812">{{cite journal |vauthors=Liu D, Chen Z, Wang T, Zhang B, Zhou H, Li Q |title=Littoral-cell angioma of the spleen: a case report |journal=Cancer Biol Med |volume=14 |issue=2 |pages=194–195 |date=May 2017 |pmid=28607812 |pmc=5444933 |doi=10.20892/j.issn.2095-3941.2016.0094 |url=}}</ref>
====Related lesions====
=====Eccrine angiomatous hamartoma=====
 
===Locally aggressive or borderline vascular tumors===
====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:
** Deep reddish-purple color
** Deep reddish-purple color
** Shiny, firm texture
** Shiny, firm texture
** Warm to the touch
** Warm to the touch
** Swollen and painful
** 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.  
* 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>
* [[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]].


====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 arborizing blood vessels are arranged in retiform pattern <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>, 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. Adjuvant radiotherapy and ddjuvant 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.  


====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.<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 interferon 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====
===Composite 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 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>
* SERPINE1-FOSB fusions are characteristic that result in over-expression of truncated form of FOSB.<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> 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>https://en.wikipedia.org/wiki/FOSB</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 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 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 includes X-ray, MRI, CT scan and biopsy of the lesion.
* [[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>
* Excision is the typical treatment but chemotherapeutic agents including gemcitabine/taxane and mammalian target of rapamycin inhibitor <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>, mTOR inhibitors such as sirolimus <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>, VEGFR1-4/PDGFRA inhibitors such as telatinib <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> have been used with success in various studies.
* [[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>


====Polymorphous hemangioendothelioma====
===Pseudomyogenic 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 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>
* 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>
* 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>
* Diagnotic work up includes histopathological examination, MRI and Ct scan.
* 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>
* Wide local excision<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> has been used for treatment, with radiation therapy in case of recurrence.<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>
* [[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>


====Kaposi sarcoma====
===Polymorphous hemangioendothelioma===
* 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.
* 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>
* There are three known variants
* 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 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===
* 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:
** 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.  
** A third form occurs in about 0.04% of kidney transplant patients. There is also a high incidence in AIDS patients.<ref>From Dorland, 27th ed and Holland et al., Cancer Medicine, 3d ed, pp2105-7</ref> 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>
** 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>
* To learn more about KS, click here.
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|click here]].


===Malignant vascular tumors===
===Epithelioid hemangioendothelioma===
====Angiosarcoma====
* 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>  
* 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>
* Usually [[asymptomatic]] but patient may present with [[respiratory]] symptoms, [[bone]] pains or other symptoms depending on the site of the [[tumor]].
* Associated with MYC gene amplification and protein overexpression.<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> Myc is a family of regulator genes and proto-oncogenes that code for transcription factors.
* 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>
* 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>
* [[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>
* To learn more about angiosarcoma click here.
* [[Surgery]] has been used as primary treatment modality depending upon the location of the [[tumor]], with [[radiotherapy]] being used in some cases.


====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==
==References==
{{Reflist|2}}
{{Reflist|2}}
{{WikiDoc Sources}}
{{WikiDoc Sources}}

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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