Targeted therapies for soft tissue sarcomas and desmoid tumors

Soft tissue sarcomas are a group of rare malignant tumors with a high recurrence and mortality rate. Little progress has been made in the treatment of this pathology over the past decades. Surgery with or without adjuvant/neoadjuvant radiotherapy is still the first-line therapy for localized soft tissue sarcomas, and chemotherapy is an additional option for high-risk patients. However, not all patients with advanced or metastatic soft tissue sarcomas benefit from conventional chemotherapy. Soft tissue sarcomas are characterized by high heterogeneity of biological behavior and a variety of genetic mutations, which indicates the perspective of personalized therapy based on tumor histotype, genetic and molecular characteristics, and new small molecule inhibitors of signaling pathways. This article provides an overview of studies on the effectiveness of targeted therapy for the most common soft tissue sarcomas and desmoid tumors.