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 hemangioma 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 the site of a previously excised atypical meningioma and in its unusually aggressive clinical course. To our knowledge this is the first report of intracranial epithelioid hemangioendothelioma with postmortem documentation. The patient initially presented with a 7 cm right post frontal tumor; an atypical meningioma was excised and removal appeared complete on immediate post-operative scans. Seven months later the patient 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 hemangioendothelioma without evidence of meningioma: both morphologically and immunohistochemically the two tumors were quite distinctive. Further recurrence prompted radiotherapy but the patient continued to deteriorate and died five months later. Massive recurrence of intracranial tumor was found at postmortem examination.