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epithelioid hemangioendothelioma

Tuesday 6 July 2004


Definition: Epithelioid hemagioendothelioma (EHE) is a rare vascular tumor with an intermediate biological behavior between hemangioma and angiosarcoma.


- Epithelioid hemagioendothelioma (EHE)

- hepatic EHE

Epithelioid hemangioendothelioma (EHE) is a rare vascular soft tissue tumor of intermediate malignancy. Although initially described as most common in the soft tissues of the extremities, other locations are possible, namely bone.

EHE was first described by Weiss and Enzinger in 1982 as a rare vascular lesion with an epithelioid appearance.

The tumor is composed of a discrete population of epithelioid endothelial cells arranged to resemble primitive capillaries with an intermediate biological behavior between hemangioma and angiosarcoma.

EH represents 1% of all vascular neoplasms and is locally aggressive.

Although initially described as being most common in the soft tissues of the extremities, other reported sites of occurrence include the liver, lung, breast, meninges, brain and bones.

There seems to be no gender predilection, although some authors consider it to be more frequent in males. The highest incidence occurs in the third and fourth decades.

Recently a rare association with neurofibromatosis type I has been


On gross pathology examination, EHE present as reddish-brown lobulated masses, well demarcated with irregular scalloped borders and a bright
red hemorrhagic appearance.


Microscopically, the tumor is characterized by anastomosing cords, solid nests, or short strands of round to slightly spindled eosinophilic neoplastic endothelial cells embedded in a chondroid-like or hyalinized stroma.

Rarely large and distinct vascular channels are identified in the center of the tumor, as contrasted with the periphery of the lesion, and mitotic activity seldom is identified in these tumors.


Immunohistochemical analysis reveals that the tumor cells are positive for vimentin and endothelial markers such as factor VIII-related antigen, ulex
europaeus lectin, CD31 and CD349.

Molecular biology

- WWTR1-CAMTA1 fusion gene

  • translocation t(1;3)(p36.3;q25) (1p36 and 3q25) (11342784)

- translocation t(7;22) (8976367)

- TFE3 rearrangements (26840265)


- hepatic epithelioid hemangioendothelioma (2596622, 6088383, 2661410)
- epithelioid hemangioendothelioma of the bone (8898834, 2430475)
- cutaneous epithelioid hemangioendothelioma (9130982)
- soft tissues epithelioid hemangioendothelioma (9130982, 8067508)
- cerebral epithelioid hemangioendothelioma (8651350)
- penile epithelioid hemangioendothelioma (15087672)
- thyroid epithelioid hemangioendothelioma (27026033)

- vascular epithelioid hemangioendothelioma

  • epithelioid hemangioendothelioma of the superior vena cava
  • epithelioid hemangioendothelioma of the inferior vena cava


- epithelioid sarcoma-like hemangioendothelioma (12502927)

Differential diagnosis

-  vascular tumors

- CD34+ tumors
- epithelioid tumors / epithelioid cell tumors
- metastasis of an atrial myxoma (8279623)

See also

- hemangioendotheliomas
- vascular tumors

Open references

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