Hemangioendothelioma: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
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{{CMG}} {{AE}} {{MV}} | {{CMG}}; {{AE}} {{MV}} | ||
{{SK}} Infantile hemangioendothelioma; Kaposiform hemangioendothelioma; Retiform hemangioendothelioma; Epithelioid hemangioendothelioma; Dabska tumor | {{SK}} Infantile hemangioendothelioma; Kaposiform hemangioendothelioma; Retiform hemangioendothelioma; Epithelioid hemangioendothelioma; Dabska tumor | ||
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==Historical Perspective== | ==Historical Perspective== | ||
*Hemangioendothelioma was first described by Sharon Weiss, an American pathologist, | *Hemangioendothelioma was first described by Dr. Sharon Weiss, MD, an American pathologist, in 1986.<ref name="wiki">Hemangioendothelioma. Wikipedia. https://en.wikipedia.org/wiki/Hemangioendothelioma Accessed on April 22, 2016</ref> | ||
==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]] | ||
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::*Type II: | ::*Type II: | ||
:::*Disorganized appearance and hypercellular | :::*Disorganized appearance and hypercellular | ||
:::* | :::*Bile ductules absent | ||
==Pathophysiology== | ==Pathophysiology== | ||
*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 mutations associated with the development of hemangioendothelioma. | ||
*On gross pathology, characteristic findings of hemangioendothelioma | *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 | *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") | :*Cytoplasmic vacuolation (some cells) (also known as "blister cells"), a key distinguishing feature | ||
:*May form lumen and | :*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 | ||
:*Arranged in an | :*Arranged in an anatomizing pattern | ||
:*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 | ||
*On immunohistochemistry, characteristic findings of hemangioendothelioma | *On immunohistochemistry, characteristic findings 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> | ||
:*Positive [[CD31]] | :*Positive [[CD31]] | ||
:*Positive [[CD34]] | :*Positive [[CD34]] | ||
| Line 62: | Line 62: | ||
==Differentiating Hemangioendothelioma from Other Diseases== | ==Differentiating Hemangioendothelioma from Other Diseases== | ||
*Hemangioendothelioma must be differentiated from other diseases that cause a hypervascular mass, or abdominal pain, | *Hemangioendothelioma must be differentiated from other diseases that cause a hypervascular mass, or [[abdominal pain]], such as:<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><ref name="radio">Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016</ref> | ||
:*[[Hepatoblastoma]] | :*[[Hepatoblastoma]] | ||
:*[[Hemangioma]] | :*[[Hemangioma]] | ||
| Line 69: | Line 69: | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
* 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=== | ||
| Line 82: | Line 81: | ||
==Risk Factors== | ==Risk Factors== | ||
*Common risk factors in the development of hemangioendothelioma | *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]] | ||
| Line 88: | Line 87: | ||
== 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 ( | *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 | *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 | ||
| Line 99: | Line 98: | ||
=== Symptoms === | === Symptoms === | ||
*Hemangioendothelioma is usually asymptomatic. | *Hemangioendothelioma is usually asymptomatic. | ||
*Symptoms of hemangioendothelioma may include | *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]] | ||
=== Physical Examination === | === Physical Examination === | ||
Revision as of 19:23, 4 August 2016
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
Hemangioendotheliomas is a rare group of mesenchymal vascular neoplasms. Hemangioendotheliomas commonly present with an enlarging mass and have been reported in the head and 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. The majority of patients with hemangioendothelioma are symptomatic at the time of diagnosis.[1] Early clinical features of hemangioendothelioma are often unspecific, such as: abdominal pain, mass, weight loss, or fatigue. On CT, characteristic findings of hepatic hemangioendothelioma, may include: multiple hypo-attenuating lesions in both hepatic lobes, 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 (with capsular retraction), and located in a predominantly peripheral. 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]
- Epithelioid hemangioendothelioma (most common)
- Kaposiform hemangioendothelioma
- Retiform hemangioendothelioma
- Infantile hemangioendothelioma
- 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
- On microscopic histopathological analysis, characteristic findings of hemangioendothelioma include:[2][3]
- Large, epithelioid, perivascular cells
- Abundant, pale, eosinophilic cytoplasm
- Cytoplasmic vacuolation (some cells) (also known as "blister cells"), a key distinguishing feature
- May form lumen and contain red blood cells
- Vesicular nucleus with prominent nucleolus in some cells
- Tuft-like projections into capillaries
Papillary intralymphatic hemangioendothelioma
- Papillary tufts
- Central hyaline core lined by hobnail-like endothelial cells protruding into the lumina
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]
- Positive CD31
- Positive CD34
- Positive Factor VIII
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
- Common risk factors in the development of hemangioendothelioma include:[1]
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]
- Hepatic failure
- Multi-organ failure
- Heart failure
- Prognosis is generally poor, and the 5-year survival rate of patients with hemangioendothelioma is approximately 55%.[4]
Diagnosis
Symptoms
- Hemangioendothelioma is usually asymptomatic.
- Symptoms of hemangioendothelioma may include:[3]
Physical Examination
- Patients with hemangioendothelioma usually may be well-appearing.
- Physical examination may be remarkable for:[4]
Palpation
- Abdominal large mass
- Hepatomegaly
Laboratory Findings
- There are no specific laboratory findings associated with hemangioendothelioma.
Imaging Findings
- On CT, characteristic findings of hemangioendothelioma, include:[4]
Hepatic Hemangioendothelioma
- Multiple hypo-attenuating lesions in both hepatic lobes
- That 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
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
Treatment
Medical Therapy
- There is no treatment for hemangioendothelioma; the mainstay of therapy is supportive care.
- Common therapies for hemangioendothelioma, may include:
- Interferon
- Sorafenib (kinase inhibitor)
- Corticosteroids
- Paclitaxel
- Thalidomide
- Doxorubicin
- Radiation therapy for hemangioendothelioma remains unclear.
Surgery
- Surgery is the mainstay of therapy for hemangioendothelioma.[3]
- The recurrence rate after surgery of hemangioendothelioma is approximately 40%.
- Infantile hepatic hemangioendothelioma has a good prognosis.[3]
Prevention
- There are no primary preventive measures available for hemangioendothelioma.
References
- ↑ 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.
- ↑ 2.0 2.1 Hemangioendothelioma. Wikipedia. https://en.wikipedia.org/wiki/Hemangioendothelioma Accessed on April 22, 2016
- ↑ 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
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 Hemangioendothelioma. Radiopedia. http://radiopaedia.org/articles/epithelioid-haemenagioendothelioma Accessed on April 22, 2016