Epithelioid hemangioendothelioma of the brain.

Abstract

Epithelioid hemangioendothelioma is a recently described vascular neoplasm characterized by epithelioid tumor cells and borderline biologic behavior. Its four principal sites of occurrence are the soft tissue, liver, lung, and bone. We report a case of primary cerebral epithelioid hemangioendothelioma in a 4-month-old male infant. The tumor consisted of loose aggregates of epithelioid cells with a focal cordlike or bridging-branching pattern, supported in a fibromyxoid stroma. The tumor cells displayed frequent intracytoplasmic vacuoles. Immunohistochemically, the tumor cells showed positive staining for Ulex europaeus agglutinin, vimentin, and cytokeratin. The tumor pursued an indolent clinical course. The patient was alive 28 months after initial presentation, but he was left with a severe neurological deficit because of the location and growth of the tumor.