Ossifying epithelioid hemangioendothelioma

Abstract

A case of small epithelioid hemangioendothelioma encapsulated by an egg-shaped bony shell composed of newly formed mature lamellar bone is presented. A nodule measuring 10 x 5 x 5 mm was excised from inside of the right cheek muscle of a 46-year-old man. Histopathologically, the nodule consisted mainly of round-to-short spindled epithelioid cells with round nuclei and occasional cytoplasmic vacuoles associated with some foci of osseous metaplasia. Characteristically, egg shell-like lamellar bone enclosed this lesion showing well-circumscribed appearance. The main component cells were immunohistochemically stained positive for factor VIII-related antigen, CD 34, UEA-1, and vimentin, and proved to be vascular endothelial cells. The term ossifying epithelioid hemangioendothelioma is proposed for this unique lesion. This type of epithelioid hemangioendothelioma has not been previously reported to our knowledge, and differentiation from ossifying fibromyxoid tumor of soft parts is considered to be important.