Soft Tissue Sarcoma

Abstract

Soft tissue sarcoma (STS) refers to a rare group of cancers that develop from mesenchymal cells and their progenitors. Histologic subtype, in conjunction with tumor location and size, largely defines the biologic behavior of a given lesion and the associated clinical prognosis in these cancers. The diverse characteristics of these tumors means that their treatment is similarly complex. The etiology, tumor staging and prognosis, evaluation, and treatment of STS are discussed in this review, with an aim to present an algorithm for patient evaluation and treatment while highlighting common indications for diverging from this strategy as dictated by disease subtype and location. Figures show the histologic distribution of primary STS diagnosed in the extremity and retroperitoneum and intra-abdominal compartments; disease-specific survival for primary extremity and retroperitoneal and intra-abdominal tumors stratified by histologic subtype; local recurrence in primary extremity STS stratified by histologic subtype; disease-specific survival according to American Joint Committee on Cancer (AJCC) TNGM stage; a postoperative nomogram for prediction of sarcoma-specific death at 12 years postresection for patients with STS; representative cross-sectional images of an atypical lipomatous tumor, a myxofibrosarcoma, and a desmoid tumor; a treatment algorithm for STS of the extremity; a magnetic resonance image and intraoperative photographs showing a mixoid liposarcoma of the posterior thigh; and computed tomography showing a retroperitoneal dedifferentiated liposarcoma and a photograph of the surgical bed following resection.  This review contains 10 figures, 12 tables, and 49 references. Keywords:  Sarcoma, soft tissue, cancer, myxofibrosarcoma, leiomyosarcoma, liposarcoma, gastrointestinal stromal tumor, rhabdomyosarcoma,