High Risk Soft Tissue Sarcoma: an Overview

Abstract

Soft tissue sarcoma is a cluster of extremely rare solid tumor malignancies. In the United States approximately 6,000 new diagnoses of this disease are rendered annually, and it constitutes less than 1% of adult solid tumors. In the pediatric age group, the overall incidence of soft tissue sarcoma is approximately 7%, where the predominate histology is rhabdomyosarcoma. The aggregate overall incidence is approximately equivalent to that of multiple myeloma or carcinoma of the salivary gland. About 10% of soft tissue sarcomas are in the head and neck area, 30% in the trunk and retroperitoneum, 15% in the upper extremity, and 45% in the lower extremity. Ten percent of the patients will present synchronous distant disease, and the pattern of relapse is primarily pulmonary. About 50–60% of patients relapse as an isolated pulmonary lesion, 30% relapse as an isolated local recurrence, and 10% relapse at other or multiple sites. In the extremity, about 75% of the failures are pulmonary, whereas in the trunk and retroperitoneum 50% of the failures are local. This points to the relative difficulty of obtaining biologically secure negative margin resections in the central body axis as compared to the extremity where amputation may be an ultimate salvage procedure. Approximately 80% of the local and distant recurrences in this disease occur within two years of initial diagnosis.