Soft-tissue sarcoma in adults: An update on the current state of histiotype-specific management in an era of personalized medicine

CA Cancer J Clin. 2020 May;70(3):200-229. doi: 10.3322/caac.21605. Epub 2020 Apr 10.

Abstract

Soft-tissue sarcomas (STS) are rare tumors that account for 1% of all adult malignancies, with over 100 different histologic subtypes occurring predominately in the trunk, extremity, and retroperitoneum. This low incidence is further complicated by their variable presentation, behavior, and long-term outcomes, which emphasize the importance of centralized care in specialized centers with a multidisciplinary team approach. In the last decade, there has been an effort to improve the quality of care for patients with STS based on anatomic site and histology, and multiple ongoing clinical trials are focusing on tailoring therapy to histologic subtype. This report summarizes the latest evidence guiding the histiotype-specific management of extremity/truncal and retroperitoneal STS with regard to surgery, radiation, and chemotherapy.

Keywords: advanced soft-tissue sarcoma; extremity sarcoma; retroperitoneal sarcoma; soft-tissue sarcoma.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Combined Modality Therapy / methods
  • Humans
  • Precision Medicine / methods*
  • Prognosis
  • Sarcoma / pathology*
  • Sarcoma / therapy
  • Soft Tissue Neoplasms / pathology*
  • Soft Tissue Neoplasms / therapy