Primary intracranial epithelioid angiosarcoma--case report.

A 39-year-old male presented with an exceedingly rare primary intracranial epithelioid angiosarcoma in the right parietal lobe manifesting as weakness of the left hand. Neuroimaging revealed a well-defined intensely enhanced lesion in the right parietal lobe with peripheral cerebral edema. The tumor was grossly totally removed. Light microscopy of the surgical specimens revealed features typical of an epithelioid vascular tumor. The tumor cells showed intense positive immunohistochemical staining for cytokeratin and vimentin and focally positive staining for both Ulex europaeus agglutinin and anti-human endothelial cells, CD31. Tumor regrowth required two further operations. This progressive growth was consistent with an angiosarcoma. The tumor was diagnosed as an epithelioid angiosarcoma based on the histological and clinical characteristics. He became progressively obtunded and finally died. This is the first intracranial epithelioid angiosarcoma which expressed epithelial markers detectable by immunohistochemical methods.