Epithelioid hemangioendothelioma of the head and neck: a clinicopathologic report of twelve cases

Abstract

Epithelioid hemangioendothelioma (EH) is a recently described unusual vascular neoplasm of the soft tissues. Apparently identical tumors have also been described in the lung and and liver. Although no previous reports have described these lesions in the head and neck, twelve cases from this region are reported here. There is a wide age distribution throughout adulthood of affected patients. The 4-year-old patient in this series is the youngest thus far reported. There is no sex predilection. The submandibular region is the head and neck site most frequently involved. The unusual histologic appearance of these tumors is demonstrated by the fact that only one case was originally diagnosed as a vascular neoplasm. Nests and cords of pleomorphic epithelioid cells, spindle cells, and vacuolated cells and a hyalinized or myxochondroid stroma are features that distinguish EH. All eight of the tumors tested showed factor VIII-related antigen immunoreactivity. EH has a biologic potential intermediate between that of hemangioma and conventional angiosarcoma.