Angiosarcoma is a rare tumor of bone that uncommonly involves the skull. Primary neoplasms of the skull represent 2.6% of primary neoplasms of bone. We wish to report a case of angiosarcoma of he skull in an 18-year-old pregnant woman who presented with a rapidly growing left frontotemporal mass. Neuroimaging revealed a left frontotemporal mass with destruction of diploic space and both tables and with extension to the subgaleal and epidural spaces. The patient underwent surgery and the tumor was removed totally. Histological features of angiosarcoma were seen in the pathology study. She received adjuvant chemotherapy and radiation therapy. She has remained well during 6 years of follow-up with no evidence of recurrence. Angiosarcoma is a rare malignant bone tumor of vascular origin. Our case is the only female patient with skull angiosarcoma that has been reported so far.
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