Primary Angiosarcoma of Bone of the Skull Base: A Case Report and Literature Review.

Primary angiosarcoma of bone (PAB) is an exceedingly rare entity characterized by aggressive clinical behavior. Following surgical removal, systemic relapse is common, and the overall prognosis is poor. Several systemic therapy agents and combinations have been used in the treatment of PAB. However, the evidence for their efficacy is limited to retrospective case series and case reports, making it difficult to assess their relative effectiveness. We report a case of systemically relapsed PAB along with a literature review aimed at increasing the available information on PAB responses to different types of systemic therapy.