Abstract

Vascular tumors are categorized into benign hemangiomas, frankly malignant angiosarcomas, and tumors of intermediate malignancy. Hemangioendotheliomas are of intermediate malignancy and encompass a broad range of histologic entities, including retiform, epithelioid, kaposiform, polymorphous, and composite sub-types [1]. Retiform hemangioendothelioma (RH) is an extremely rare disease entity of unknown etiology that was first described by Calonje et al. [2] in 1994 as a vascular neoplasm of intermediate malignancy. It is characterized by a high rate of local recurrence and a low frequency of metastasis, and its biologic potential is between that of hemangiomas and angiosarcomas. RH reportedly presents primarily as an exophytic dermal tumor of the extremities [3]. We present the first report of RH arising in the neck without dermal involvement.

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