More Than 50 Subtypes of Soft Tissue Sarcoma: Paving the Path for Histology-Driven Treatments

Abstract

Sarcomas are a diverse group of cancers with mesenchymal origin. Although sarcomas comprise less than 1% of cancers, there are more than 50 different subtypes that are quite different from one another in terms of both their biology and clinical behavior. Historically, the need for adequate patient numbers in clinical trials has pushed sarcoma researchers to lump these very different malignancies together and treat the patients using a “one-size-fits-all” approach. However, with improvements in our scientific understanding, we are finally ready for a histology-tailored therapeutic approach to these complex diseases. In this review, we discuss key advances in our understanding of the biology underlying selected sarcoma subtypes and how targeting these subtypes is relevant therapeutically with respect to both molecularly targeted agents as well as immunotherapy.