Soft Tissue Sarcoma

Soft tissue sarcoma (STS) is a relatively rare malignancy. The median age of onset of STS is from 50 to 55 years of age. Deep lesions with history of growth or any superficial/deep abnormalities with prior radiation exposure require careful evaluation. Approximately 50% arise in the extremities, 40% in the trunk and retroperitoneum, and 10% in the head and neck. STS are characterized by wide heterogeneity in anatomical site, pathologic subtype, and clinical behavior. Histologic confirmation is necessary, with careful planning of initial core-needle or incisional biopsy to avoid compromising curative resection. Prebiopsy cross-sectional imaging (computed tomography [CT] and/or magnetic resonance imaging [MRI]) of primary lesion should be done. Prognosis depends on a patient’s age, and the size, histologic grade, and stage of the tumor. Review of biopsy tissue, staging, and treatment planning should be undertaken by a multidisciplinary team of specialists experienced in sarcoma management. Low-grade, superficial, small tumors (<5 cm) are usually curable by surgery alone. High-grade tumors usually require a combined-modality approach of preoperative or postoperative radiation therapy in combination with function-preserving surgery. Role of chemotherapy is less well defined. Anthracycline-based chemotherapy is the mainstay of adjuvant and metastatic regimes. The most commonly observed metastatic site is lung. Treatment of metastatic disease includes surgery for patients with optimal underlying biologic behavior and pulmonary metastases. Gastrointestinal stromal tumors are a different entity and require a different management approach with surgery and molecularly-targeted therapies.