Retroperitoneal Sarcomas: Histology Is Everything

Abstract

Retroperitoneal sarcomas (RPS) are a rare subset of soft tissue sarcoma that are composed of only a few histologic subtypes, each with a distinct tumor biology, clinical presentation, preferred treatment strategy, recurrence risk, and surveillance plan. In the modern era of precision medicine, our understanding of the implications of subtype tumor biology and anatomic location has led to a more nuanced, histology-specific approach to therapy, including surgery, neoadjuvant radiation therapy, and/or chemotherapy. This article provides a summary of recent updates to the management of RPS.

Keywords: Active surveillance; Histiotype; Multimodality therapy; Retroperitoneal sarcoma; Surgery.