Lessons learned from the study of soft tissue sarcoma.

Soft tissue sarcoma is a rare disease. We began a prospective database for in-patients treated on the surgical service at Memorial Sloan-Kettering in 1982. We now celebrate 30 years of this database which has been reviewed and cataloged constantly on a weekly basis. We approach 10,000 treated patients. The study of this heterogeneous group of diverse pathology rising in the soft tissue has been fascinating. We have learned about demographics, prevalence, incidence, and have recognized the value of such databases in describing not just outcome but biology. Site is an important determinant of outcome and illustrates the complexities of including all sites in any staging system. For example, local recurrence in the extremity, while associated with a decrease in survival, is clearly not causative. Conversely, retroperitoneal sarcoma, particularly liposarcoma, is a common locally recurring disease with local progression often the cause of disease-specific mortality. Genetic predispositions have been defined. Radiation therapy and lymphedema, both alone and in combination, predispose to the development of sarcoma. These observations have important relevance as we increasingly utilize radiation therapy to minimize local recurrence in early stage breast cancer and ductal carcinoma in situ. It is clear that outcome is histology specific and wide variations in outcome, depending on the underlying histological types and subtypes, are evident. We have established very clearly that limb preservation as opposed to amputation is equally efficacious and does not diminish survival. Radiation therapy can limit local recurrence but must be balanced against side effects. Unfortunately, the majority of chemotherapeutic regimens have had minimal benefit.