INTRACRANIAL EPITHELIOID HEMANGIOENDOTHELIOMA ARISING AT SITE OF PREVIOUSLY EXCISED ATYPICAL MENINGIOMA

Epithelioid hemangioendothelioma is an uncommon vascular tumor which, in most cases, pursues a clinical course intermediate between hemangio ma and angiosarcoma. Only four completely documented cases of central nervous system involvement by this tumor appear in the literature. We present an additional case, which is remarkable in its occurrence at t he site of a previously excised atypical meningioma and in its unusual ly aggressive clinical course. To our knowledge this is the first repo rt of intracranial epithelioid hemangioendothelioma with postmortem do cumentation. The patient initially presented with a 7 cm right post fr ontal tumor; an atypical meningioma was excised and removal appeared c omplete on immediate post-operative scans. Seven months later the pati ent re-presented with tumor recurrence at the previous operative site; a second craniotomy was performed and, on this occasion, the excised tumor had the histological features of an epithelioid hemangioendothel ioma without evidence of meningioma: both morphologically and immunohi stochemically the two tumors were quite distinctive. Further recurrenc e prompted radiotherapy but the patient continued to deteriorate and d ied five months later. Massive recurrence of intracranial tumor was fo und at postmortem examination.