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==Overview==
==Overview==
Hemangioendothelioma is a rare group of mesenchymal vascular neoplasms. Hemangioendotheliomas commonly present with an enlarging mass and have been reported in the [[head]], [[neck]], [[lungs]], [[lymph nodes]], [[pleura]], [[retroperitoneum]], or [[stomach]]. Hemangioendotheliomas may be [[benign]] or [[malignant]] and tend to occur around medium to large venous structures. Hemangioendothelioma may be classified into 4 groups: epitheloid hemangioendothelioma (most common), [[kaposiform hemangioendothelioma]], retiform hemangioendothelioma, and [[infantile hemangioendothelioma]]. The prevalence of hemangioendothelioma is approximately 1 per 100,000 individuals worldwide.
Hemangioendothelioma is a rare group of mesenchymal vascular neoplasms. Hemangioendotheliomas commonly present with an enlarging mass and have been reported in the [[head]], [[neck]], [[lungs]], [[lymph nodes]], [[pleura]], [[retroperitoneum]], or [[stomach]]. Hemangioendotheliomas may be [[benign]] or [[malignant]] and tend to occur around medium to large [[venous]] structures. Hemangioendothelioma may be classified into 4 groups including epitheloid hemangioendothelioma (most common), [[kaposiform hemangioendothelioma]], retiform hemangioendothelioma, and [[infantile hemangioendothelioma]]. The prevalence of hemangioendothelioma is approximately 1 per 100,000 individuals worldwide.
The majority of patients with hemangioendothelioma are symptomatic at the time of diagnosis.<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref> Early clinical features of hemangioendothelioma are often unspecific and may include [[abdominal pain]], mass, [[weight loss]], or [[fatigue]]. On CT, characteristic findings of hepatic hemangioendothelioma may include multiple hypo-attenuating lesions in both hepatic lobes which coalesce to form larger confluent hypo-attenuating regions in a peripheral or subcapsular distribution, halo or target pattern of enhancement in larger lesions, and a subcapsular lesion (with capsular retraction). Surgical [[resection]], [[radiotherapy]], and [[chemotherapy]] are often the treatment of choice for hemangioendothelioma.
The majority of patients with hemangioendothelioma are symptomatic at the time of diagnosis.<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref> Early clinical features of hemangioendothelioma are often unspecific and may include [[abdominal pain]], mass, [[weight loss]], or [[fatigue]]. On [[CT scan]], characteristic findings of [[hepatic]] hemangioendothelioma may include multiple hypo-attenuating lesions in both hepatic lobes which coalesce to form larger confluent hypo-attenuating regions in a peripheral or subcapsular distribution, halo or target pattern of enhancement in larger lesions, and a subcapsular lesion (with capsular retraction). Surgical [[resection]], [[radiotherapy]], and [[chemotherapy]] are often the treatment of choice for hemangioendothelioma.


==Historical Perspective==
==Historical Perspective==
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==Classification==
==Classification==
*Hemangioendothelioma may be classified into 4 groups:<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref>
*Hemangioendothelioma may be classified into 4 groups:<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref>
:*Epithelioid hemangioendothelioma (most common)  
 
:*Epithelioid hemangioendothelioma (most common)
:*[[Kaposiform hemangioendothelioma]]
:*[[Kaposiform hemangioendothelioma]]
:*Retiform hemangioendothelioma
:*Retiform hemangioendothelioma
:*[[Infantile hemangioendothelioma]]
:*[[Infantile hemangioendothelioma]]
::*Type I
::*Type I
:::*Multiple vascular channels  
:::*Multiple vascular channels
:::*Formed by an immature endothelial lining
:::*Formed by an immature endothelial lining
:::*Stromal separation from bile ductules
:::*[[Stromal]] separation from bile ductules
::*Type II:  
::*Type II:  
:::*Disorganized appearance and hypercellular
:::*Disorganized appearance and hypercellular
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*The pathogenesis of hemangioendothelioma is characterized by [[epithelioid]]-like features.<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref>
*The pathogenesis of hemangioendothelioma is characterized by [[epithelioid]]-like features.<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref>
*Hemangioendotheliomas tend to occur around medium to large [[venous]] structures.<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref>
*Hemangioendotheliomas tend to occur around medium to large [[venous]] structures.<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref>
*There are no genetic mutations associated with the development of hemangioendothelioma.
*There are no genetic [[Mutation|mutations]] associated with the development of hemangioendothelioma.
*On gross pathology, characteristic findings of hemangioendothelioma include:  
*On gross pathology, characteristic findings of hemangioendothelioma include:  
:*No hallmark features
:*No hallmark features
:*Size may range up to 18 cm  
:*Size may range up to 18 cm  
*On microscopic histopathological analysis, characteristic findings of hemangioendothelioma include:<ref name="wiki">Hemangioendothelioma. Wikipedia. https://en.wikipedia.org/wiki/Hemangioendothelioma Accessed on April 22, 2016</ref><ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref>
*On microscopic [[histopathological]] analysis, characteristic findings of hemangioendothelioma include:<ref name="wiki">Hemangioendothelioma. Wikipedia. https://en.wikipedia.org/wiki/Hemangioendothelioma Accessed on April 22, 2016</ref><ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref>
 
:*Large, [[epithelioid]], perivascular cells
:*Large, [[epithelioid]], perivascular cells
:*Abundant, pale, [[eosinophilic]] cytoplasm
:*Abundant, pale, [[eosinophilic]] [[cytoplasm]]
:*Cytoplasmic vacuolation (some cells) (also known as "blister cells"), a key distinguishing feature
:*[[Cytoplasmic]] vacuolation (some cells) (also known as "blister cells"), a key distinguishing feature
:*May form lumen and contain [[red blood cells]]
:*May form [[lumen]] and contain [[red blood cells]]
:*Vesicular nucleus with prominent [[nucleolus]] in some cells
:*Vesicular nucleus with prominent [[nucleolus]] in some cells
:*Tuft-like projections into capillaries
:*Tuft-like projections into [[capillaries]]
 
'''Papillary intralymphatic hemangioendothelioma'''
'''Papillary intralymphatic hemangioendothelioma'''
:*[[Papillary]] tufts
:*[[Papillary]] tufts
:*Central hyaline core lined by hobnail-like endothelial cells protruding into the lumina
:*Central [[hyaline]] core lined by hobnail-like [[endothelial cells]] protruding into the lumina
'''Retiform hemangioendothelioma'''
'''Retiform hemangioendothelioma'''
:*Infiltrative [[neoplasm composed]] of elongated, arborizing vessels
:*Infiltrative [[neoplasm composed]] of elongated, arborizing vessels
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:*Lined by a single layer of "hobnail-like" endothelial cells (protrude within the narrow lumina)
:*Lined by a single layer of "hobnail-like" endothelial cells (protrude within the narrow lumina)
'''Kaposiform hemangioendothelioma'''
'''Kaposiform hemangioendothelioma'''
:*Composed of several solid, poorly circumscribed nodules
 
:*Composed of several solid, poorly circumscribed [[nodules]]
::*Nodules composed of a mixture of small capillaries and solid lobules
::*Nodules composed of a mixture of small capillaries and solid lobules
:*Arranged in a glomeruloid pattern
:*Arranged in a glomeruloid pattern
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==Epidemiology and Demographics==
==Epidemiology and Demographics==
*Hemangioendothelioma is very rare. The prevalence of hemangioendothelioma is approximately 1 per 100,000 individuals worldwide.<ref name="radio">Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016</ref>
*Hemangioendothelioma is very rare. The [[prevalence]] of hemangioendothelioma is approximately 1 per 100,000 individuals worldwide.<ref name="radio">Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016</ref>
   
   
===Age===
===Age===
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==Risk Factors==
==Risk Factors==
*Common risk factors in the development of hemangioendothelioma include:<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref>
*Common [[risk factors]] in the development of hemangioendothelioma include:<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref>
:*[[Oral contraceptive|Oral contraceptives]]
:*[[Oral contraceptive|Oral contraceptives]]
:*[[Polyvinyl chloride]]
:*[[Polyvinyl chloride]]


== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*The majority of patients with hemangioendothelioma are symptomatic at the time of diagnosis.<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref>
*The majority of patients with hemangioendothelioma are symptomatic at the time of [[diagnosis]].<ref name="pmid3303234">{{cite journal |vauthors=Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM |title=Epithelioid hemangioendothelioma and related lesions |journal=Semin Diagn Pathol |volume=3 |issue=4 |pages=259–87 |year=1986 |pmid=3303234 |doi= |url=}}</ref>
*Early clinical features of hemangioendothelioma are often unspecific (e.g., [[abdominal pain]], mass, [[weight loss]], or [[fatigue]]).
*Early clinical features of hemangioendothelioma are often unspecific (e.g., [[abdominal pain]], mass, [[weight loss]], or [[fatigue]]).
*If left untreated, the majority of patients with hemangioendothelioma may progress to develop [[metastases]].  
*If left untreated, the majority of patients with hemangioendothelioma may progress to develop [[metastases]].  
*Common complications of hemangioendothelioma include:<ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref>
*Common [[complications]] of hemangioendothelioma include:<ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref>
:*[[Hepatic failure]]
:*[[Hepatic failure]]
:*Multi-organ failure
:*Multi-organ failure
:*[[Heart failure]]  
:*[[Heart failure]]  
*Prognosis is generally poor, and the 5-­year survival rate of patients with hemangioendothelioma is approximately 55%.<ref name="radio">Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016</ref>
*Prognosis is generally poor, and the 5-­year [[survival rate]] of patients with hemangioendothelioma is approximately 55%.<ref name="radio">Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016</ref>
**Infantile hepatic hemangioendothelioma has a good prognosis.<ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref>
**Infantile hepatic hemangioendothelioma has a good [[prognosis]].<ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref>


== Diagnosis ==
== Diagnosis ==
=== Symptoms ===
=== Symptoms ===
*Hemangioendothelioma is usually asymptomatic.
*Hemangioendothelioma is usually [[asymptomatic]].
*Symptoms of hemangioendothelioma may include:<ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref>
*[[Symptoms]] of hemangioendothelioma may include:<ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref>
:*[[Abdominal pain]]
:*[[Abdominal pain]]
:*[[Fatigue]]
:*[[Fatigue]]
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=== Physical Examination ===
=== Physical Examination ===
*Patients with hemangioendothelioma usually may be well-appearing.
*Patients with hemangioendothelioma usually may be well-appearing.
*Physical examination may be remarkable for:<ref name="radio">Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016</ref>
*[[Physical examination]] may be remarkable for:<ref name="radio">Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016</ref>
:*[[Palpation]]
:*[[Palpation]]
:*Large [[abdominal mass]]
:*Large [[abdominal mass]]
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===Imaging Findings===
===Imaging Findings===
*On x ray, characteristic findings of hemangioendothelioma include nonspecific soft tissue mass.  
*On [[X-ray]], characteristic findings of hemangioendothelioma include nonspecific soft tissue mass.
:*When involving bone, hemangioendotheliomas are predominantly [[lytic]] and may mimic hemangiomas with a course honeycomb appearance
:*When involving bone, hemangioendotheliomas are predominantly [[lytic]] and may mimic hemangiomas with a course honeycomb appearance


*On CT, characteristic findings of hemangioendothelioma include:<ref name="radio">Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016</ref>
*On [[CT scan]], characteristic findings of hemangioendothelioma include:<ref name="radio">Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016</ref>
:'''Hepatic Hemangioendothelioma'''
:'''Hepatic Hemangioendothelioma'''
*Nonspecific soft tissue mass with density similar to muscle, but demonstrates bright contrast enhancement
*Nonspecific soft tissue mass with [[density]] similar to muscle, but demonstrates bright [[contrast]] enhancement.
 
:*Multiple hypo-attenuating lesions in both hepatic lobes
:*Multiple hypo-attenuating lesions in both hepatic lobes
:*Lesions coalesce to form larger confluent hypo-attenuating regions in a peripheral or subcapsular distribution  
:*Lesions coalesce to form larger confluent hypo-attenuating regions in a peripheral or subcapsular distribution  
:*Halo or target pattern of enhancement in larger lesions
:*Halo or target pattern of enhancement in larger lesions
:*Subcapsular lesion often present with capsular retraction
:*Subcapsular lesion often present with capsular [[retraction]]
:*Located in a predominantly peripheral distribution, with coalescence as individual nodules
:*Located in a predominantly peripheral distribution, with coalescence as individual nodules
:*Honeycomb appearance
:*Honeycomb appearance


*On MRI, characteristic findings of hemangioendothelioma include:<ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref><ref name="radio">Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016</ref>
*On [[MRI]], characteristic findings of hemangioendothelioma include:<ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref><ref name="radio">Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016</ref>
:'''Hepatic Hemangioendothelioma'''
:'''Hepatic Hemangioendothelioma'''
:*T1: hypointense lesions relative to normal liver parenchyma on unenhanced T1-weighted images
:*T1: hypointense lesions relative to normal liver [[parenchyma]] on unenhanced T1-weighted images
:*T2: heterogeneously increased signal intensity
:*T2: heterogeneously increased signal intensity
:*C+ (Gd): some lesions demonstrate either a peripheral halo or a target-type enhancement pattern after administration of a gadolinium-based contrast agent, with occasional observation of a thin peripheral hypointense rim
:*C+ (Gd): some lesions demonstrate either a peripheral halo or a target-type enhancement pattern after administration of a [[gadolinium]]-based contrast agent, with occasional observation of a thin peripheral hypointense rim


*On angiography, characteristic findings of hemangioendothelioma include dense, well-circumscribed areas of enhancement with early draining veins and shunting.
*On [[angiography]], characteristic findings of hemangioendothelioma include dense, well-circumscribed areas of enhancement with early draining [[Vein|veins]] and shunting.


== Treatment ==
== Treatment ==
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:*[[Thalidomide]]
:*[[Thalidomide]]
:*[[Doxorubicin]]
:*[[Doxorubicin]]
*Radiation therapy for hemangioendothelioma remains unclear.  
*[[Radiation therapy]] for hemangioendothelioma remains unclear.


=== Surgery ===
=== Surgery ===
*Surgery is the mainstay of therapy for hemangioendothelioma.<ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref>
*Surgery is the mainstay of [[therapy]] for hemangioendothelioma.<ref name="wjkjki">Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016</ref>
*The recurrence rate after surgery of hemangioendothelioma is approximately 40%.
*The recurrence rate after [[surgery]] of hemangioendothelioma is approximately 40%.


=== Prevention ===
=== Prevention ===

Latest revision as of 16:01, 23 October 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Infantile hemangioendothelioma; Kaposiform hemangioendothelioma; Retiform hemangioendothelioma; Epithelioid hemangioendothelioma; Dabska tumor

Overview

Hemangioendothelioma is a rare group of mesenchymal vascular neoplasms. Hemangioendotheliomas commonly present with an enlarging mass and have been reported in the head, neck, lungs, lymph nodes, pleura, retroperitoneum, or stomach. Hemangioendotheliomas may be benign or malignant and tend to occur around medium to large venous structures. Hemangioendothelioma may be classified into 4 groups including epitheloid hemangioendothelioma (most common), kaposiform hemangioendothelioma, retiform hemangioendothelioma, and infantile hemangioendothelioma. The prevalence of hemangioendothelioma is approximately 1 per 100,000 individuals worldwide. The majority of patients with hemangioendothelioma are symptomatic at the time of diagnosis.[1] Early clinical features of hemangioendothelioma are often unspecific and may include abdominal pain, mass, weight loss, or fatigue. On CT scan, characteristic findings of hepatic hemangioendothelioma may include multiple hypo-attenuating lesions in both hepatic lobes which coalesce to form larger confluent hypo-attenuating regions in a peripheral or subcapsular distribution, halo or target pattern of enhancement in larger lesions, and a subcapsular lesion (with capsular retraction). Surgical resection, radiotherapy, and chemotherapy are often the treatment of choice for hemangioendothelioma.

Historical Perspective

  • Hemangioendothelioma was first described by Dr. Sharon Weiss, MD, an American pathologist, in 1986.[2]

Classification

  • Hemangioendothelioma may be classified into 4 groups:[1]
  • Type I
  • Multiple vascular channels
  • Formed by an immature endothelial lining
  • Stromal separation from bile ductules
  • Type II:
  • Disorganized appearance and hypercellular
  • Bile ductules absent

Pathophysiology

  • The pathogenesis of hemangioendothelioma is characterized by epithelioid-like features.[1]
  • Hemangioendotheliomas tend to occur around medium to large venous structures.[1]
  • There are no genetic mutations associated with the development of hemangioendothelioma.
  • On gross pathology, characteristic findings of hemangioendothelioma include:
  • No hallmark features
  • Size may range up to 18 cm

Papillary intralymphatic hemangioendothelioma

Retiform hemangioendothelioma

  • Infiltrative neoplasm composed of elongated, arborizing vessels
  • Arranged in an anatomizing pattern
  • Lined by a single layer of "hobnail-like" endothelial cells (protrude within the narrow lumina)

Kaposiform hemangioendothelioma

  • Composed of several solid, poorly circumscribed nodules
  • Nodules composed of a mixture of small capillaries and solid lobules
  • Arranged in a glomeruloid pattern
  • On immunohistochemistry, characteristic findings of hemangioendothelioma include:[1]

Causes

  • There are no established causes for hemangioendothelioma.[3]

Differentiating Hemangioendothelioma from Other Diseases

  • Hemangioendothelioma must be differentiated from other diseases that cause a hypervascular mass, or abdominal pain, such as:[1][4]

Epidemiology and Demographics

  • Hemangioendothelioma is very rare. The prevalence of hemangioendothelioma is approximately 1 per 100,000 individuals worldwide.[4]

Age

  • Patients of all age groups may develop hemangioendothelioma.

Gender

  • Hemangioendothelioma affects men and women equally.

Race

  • There is no racial predilection for hemangioendothelioma.

Risk Factors

Natural History, Complications and Prognosis

  • The majority of patients with hemangioendothelioma are symptomatic at the time of diagnosis.[1]
  • Early clinical features of hemangioendothelioma are often unspecific (e.g., abdominal pain, mass, weight loss, or fatigue).
  • If left untreated, the majority of patients with hemangioendothelioma may progress to develop metastases.
  • Common complications of hemangioendothelioma include:[3]
  • Prognosis is generally poor, and the 5-­year survival rate of patients with hemangioendothelioma is approximately 55%.[4]
    • Infantile hepatic hemangioendothelioma has a good prognosis.[3]

Diagnosis

Symptoms

Physical Examination

Laboratory Findings

  • There are no specific laboratory findings associated with hemangioendothelioma.

Imaging Findings

  • On X-ray, characteristic findings of hemangioendothelioma include nonspecific soft tissue mass.
  • When involving bone, hemangioendotheliomas are predominantly lytic and may mimic hemangiomas with a course honeycomb appearance
  • On CT scan, characteristic findings of hemangioendothelioma include:[4]
Hepatic Hemangioendothelioma
  • Nonspecific soft tissue mass with density similar to muscle, but demonstrates bright contrast enhancement.
  • Multiple hypo-attenuating lesions in both hepatic lobes
  • Lesions coalesce to form larger confluent hypo-attenuating regions in a peripheral or subcapsular distribution
  • Halo or target pattern of enhancement in larger lesions
  • Subcapsular lesion often present with capsular retraction
  • Located in a predominantly peripheral distribution, with coalescence as individual nodules
  • Honeycomb appearance
  • On MRI, characteristic findings of hemangioendothelioma include:[3][4]
Hepatic Hemangioendothelioma
  • T1: hypointense lesions relative to normal liver parenchyma on unenhanced T1-weighted images
  • T2: heterogeneously increased signal intensity
  • C+ (Gd): some lesions demonstrate either a peripheral halo or a target-type enhancement pattern after administration of a gadolinium-based contrast agent, with occasional observation of a thin peripheral hypointense rim
  • On angiography, characteristic findings of hemangioendothelioma include dense, well-circumscribed areas of enhancement with early draining veins and shunting.

Treatment

Medical Therapy

  • There is no treatment for hemangioendothelioma; the mainstay of therapy is supportive care.
  • Common therapies for hemangioendothelioma may include:

Surgery

  • Surgery is the mainstay of therapy for hemangioendothelioma.[3]
  • The recurrence rate after surgery of hemangioendothelioma is approximately 40%.

Prevention

  • There are no primary preventive measures available for hemangioendothelioma.

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM (1986). "Epithelioid hemangioendothelioma and related lesions". Semin Diagn Pathol. 3 (4): 259–87. PMID 3303234.
  2. ↑ 2.0 2.1 Hemangioendothelioma. Wikipedia. https://en.wikipedia.org/wiki/Hemangioendothelioma Accessed on April 22, 2016
  3. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Hemangioendothelioma. Libre Pathology. https://librepathology.org/wiki/Infantile_hepatic_hemangioendothelioma Accessed on April 22, 2016
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016
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