Clinician's primer for soft tissue sarcomas: Nuances of histologic subtypes

Abstract

Soft tissue sarcomas are a rare group of mesenchymal malignancies, with greater than 100 histologic subtypes. Advancements in understanding these subtypes has enabled histology-tailored management. This primer describes the workup and management of generalized soft tissue sarcomas of the extremity, trunk, and retroperitoneum while also highlighting the unique attributes of many subtypes. The subtypes chosen for review include those that are most common as well as those demonstrating unique behaviors or targets for management. The focus is on initial management of localized disease; however, for situations in which novel systemic agents have been discovered, the treatment of metastatic disease is discussed. This report is a reference to be used in addition to other comprehensive reviews, such as guidelines from the National Comprehensive Cancer Network, the European Society for Medical Oncology, and the American Society for Radiation Oncology. It is not a substitute for referral to an expert sarcoma center for critical pathology review and management by an experienced team. Importantly, patients who are treated at expert sarcoma centers have better outcomes than those who are not.