Epithelioid hemangioendothelioma: A rare vascular tumor

Abstract

Epithelioid hemangioendothelioma is an intermediate-grade vascular tumor arising from the vascular endothelium, which usually arises in soft tissue, and skin involvement is extremely rare. We report a case that presented with primary cutaneous tumor involving the whole limb and was present since birth.

Oral Epithelioid Hemangioendothelioma — Unusual Location of a Rare Entity

“…2,3 The clinical features of EHE include a solitary mass localized either superficial or deep, asymptomatic, with poorly delimited contour, infiltrating the adjacent structures. 5,8 Sometimes the tumor may show multifocal or metastatic behavior. The case presented here was painless, with no important complaint except for a slight deformation of the palate and persistent 'ulcerative stomatitis'.…”

“…The tumor is usually located in the soft tissue of the extremities and less frequently in the liver, lung, pleura, bones, skin, lymph nodes, and central nervous system. 2,[5][6][7] EHE is rarely diagnosed in the head and neck region, and even more rarely in the oral cavity. 2,3 The World Health Organization (WHO) classifies EHE as a locally aggressive tumor with metastatic potential; the clinical and histological behavior is intermediate between hemangioma and angiosarcoma.…”

“…2,3 The World Health Organization (WHO) classifies EHE as a locally aggressive tumor with metastatic potential; the clinical and histological behavior is intermediate between hemangioma and angiosarcoma. [1][2][3][4][5][6][7][8] A series of histologic types of hemangioendothe-lioma were described: kaposiform, Dabska-retiform (or hobnail), compound or complex, low grade polymorphic and epithelioid, the latter being considered the most aggressive type, with high metastatic potential. 7 EHE is typically found as an asymptomatic solitary mass, with poorly defined infiltrative borders and painless growth.…”

Cutaneous Epithelioid Hemangioendothelioma on the Sole of a Child

“…In the skin, it seems that this neoplasm usually arises from a medium‐sized or large vein, mainly in the superficial or deep soft tissue, and only approximately 10% of the cases arise from superficial dermal vessels of the skin or mucous membranes . Although the clinical presentation of EHE may be variable, the neoplasm in the skin usually presents as a solitary, rarely multiple, erythematous papule, nodule, plaque, or hyperkeratotic or nonhealing ulcer that may subsequently extend to the underlying tissues, including the bone.…”

“…Epithelioid hemangioendothelioma (EHE) is a low‐grade, malignant, vascular neoplasm first described in 1982 by Weiss and Enzinger . It mostly presents as a solitary, slightly painful soft‐tissue tumor, although it has also been identified in the skin . EHEs occur without sex preference and at any age, although most described cases developed in adults (ages 30–50 yrs), and they are extremely uncommon during childhood, with only five infantile cases reported to date .…”