Rapid Clinical Course of Multiple Metastatic Cerebral Angiosarcoma

Central nervous system (CNS) angiosarcoma, both primary and secondary, is an exceptionally rare malignant tumor. The behaviour of angiosarcoma is one of rapid progression with early metastatic spread but as with all sarcomas, the primary site may not clinically evident by the time metastases develop. Identification of the primary site of origin is masked by the carpet-like growth pattern that may occur in the left atrium, the most frequently encountered primary site for angiosarcoma. This rare tumor presents many diagnostic challenges. We describe the clinical and neuropathologic features of two patients with angiosarcoma of the brain and review the previously published cases described to date.