Primary central nervous system angiosarcoma with recurrent acute subdural hematoma

Angiosarcoma is an infrequent tumor among sarcomas, especially presenting as a primary tumor within the central nervous system, which can lead to a rapid neurological deterioration and death in few months. We present a 41-year old man with a right frontal enhancing hemorrhagic lesion. Surgery was performed with histopathological findings suggesting a primary central nervous system angiosarcoma. He was discharged uneventfully and received adjuvant chemotherapy and radiotherapy. At 5 months, the follow-up MRI showed two lesions with an acute subdural hematoma, suggesting a relapse. Surgery was again conducted finding tumoral membranes attached to the internal layer of the duramater around the right hemisphere. The patient died a few days later due to the recurrence of the subdural hematoma. This case report illustrates a rare and lethal complication of an unusual tumor. The literature reviewed shows that gross-total resection with adjuvant radiotherapy seems to be the best treatment of choice.