Soft tissue sarcoma

Soft tissue sarcomas (STS) are a heterogeneous group of rare malignant tumors that arise from mesenchymal (connective) tissues, including muscle, fat, fibrous tissue, blood vessels, nerves, and other supportive tissues of the body. They can occur virtually anywhere in the body but are most commonly found in the extremities (arms and legs), trunk, retroperitoneum (the area behind the abdominal cavity), and head and neck region. There are more than 50 distinct histological subtypes, including leiomyosarcoma, liposarcoma, undifferentiated pleomorphic sarcoma, synovial sarcoma, fibrosarcoma, angiosarcoma, and rhabdomyosarcoma, among others. Symptoms vary depending on the location and size of the tumor but often include a painless or mildly painful growing mass or swelling. As tumors enlarge, they may compress surrounding structures, causing pain, restricted movement, or organ dysfunction. Soft tissue sarcomas can occur at any age but are more common in adults, with certain subtypes (such as rhabdomyosarcoma) being more prevalent in children. Most cases arise sporadically, though certain genetic predisposition syndromes increase risk, including Li-Fraumeni syndrome (TP53 mutations), neurofibromatosis type 1 (NF1 mutations), familial adenomatous polyposis (Gardner syndrome), and hereditary retinoblastoma (RB1 mutations). Environmental risk factors include prior radiation therapy and exposure to certain chemicals. Diagnosis typically involves imaging studies (MRI, CT) and tissue biopsy with histopathological and molecular analysis for accurate subtyping. Treatment depends on the tumor type, grade, stage, and location. Surgical resection with wide margins remains the cornerstone of therapy for localized disease. Radiation therapy is frequently used as an adjunct to surgery to reduce local recurrence risk. Chemotherapy, including agents such as doxorubicin and ifosfamide, is used for advanced or metastatic disease, though response rates vary by subtype. Targeted therapies, including pazopanib and trabectedin, have been approved for certain subtypes. Immunotherapy is an emerging area of investigation. Despite advances, metastatic soft tissue sarcoma carries a guarded prognosis, and management ideally occurs at specialized sarcoma centers with multidisciplinary expertise.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Soft tissue sarcoma