Soft tissue sarcomas: Current trends in diagnosis and management

Abstract

Sarcomas (fleshy tumors) were distinguished from carcinoma (crab leg tumors) at the time of Hippocrates. Sarcomas are related embryologically to leukemias and lymphomas because all are though to be malignancies of mesodermal embryologic origin. Neurosarcomas, however, are an exception, since they arise from tissue of neuroepidermal origin. Malignant sarcomas of the soft tissue represent an unusual primary malignant tumor. These lesions are characterized by their diversity in the histologic appearance and in their biologic behavior, as well as in anatomical origin. Currently there are approximately 7,400 newly diagnosed bone and soft tissue sarcomas, and approximately 4,200 deaths per year in the United States. The incidence of sarcomas varies by histologic type in various age groups. Embryonal rhabdomyosarcoma in the orbit peaks in the 4-year-old, and in the urinary tract in adolescence. Osteosarcoma has peak incidence in the teenage years, and Ewing's sarcoma develops between the ages of 15 and 30. Other sarcomas such as malignant fibrous histiocytoma and chondrosarcoma generally occur in patients aged more than 55 years. The incidence of osteosarcoma in whites and nonwhites is equal; Ewing's sarcoma is predominantly a disease of Caucasians. The relatively infrequent occurrence of these tumors plus their diverse histology and diverse presentations have made it difficult for any one institution to have enough patients to directly compare, in a randomized prospective fashion, one treatment with another in order to determine the optimal primary therapy. The purpose of this monograph is to review recent concepts in terms of pathology, surgery, radiation therapy, chemotherapy, and multimodality therapy.