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

## TL;DR

This paper presents a case of aggressive bone cancer called primary angiosarcoma of bone and reviews existing treatments and outcomes.

## Contribution

The study adds a new case report and literature review to better understand systemic therapy responses in PAB.

## Key findings

- PAB is rare and has a poor prognosis despite surgical removal.
- Systemic relapse is common, and treatment effectiveness is unclear due to limited evidence.
- The case and review highlight the need for more research on systemic therapies for PAB.

## Abstract

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.