Soft tissue sarcomas (STS) are uncommon malignancies, arising in about 11,410 persons in the United States each year and accounting for 4390 deaths, mostly due to either locoregional recurrence or distant metastasis (1,2,3,4). Although malignant tumors of soft tissue are scarce, benign tumors such as lipomas are 100 times more common. STS occur at any age with a median age of around 50-years old, and are equally common in men and women.
STS constitute a highly heterogeneous group of tumors with respect to anatomical distribution, histologic subtype, and clinical behavior (1). STS occur throughout the body, but nearly one-half occur in the extremities, with about one-third occurring in the lower extremity and 15% occurring in the upper extremity. Another one-third of STS occur in the abdomen, and these are equally divided among intra-abdominal visceral sarcomas (primarily gastrointestinal stromal tumors and leiomyosarcomas) and retroperitoneal sarcomas. Other anatomic sites include the head/neck, trunk, and other miscellaneous sites (e.g., heart).
STS are malignant tumors which arise from the mesodermal tissues (e.g., fat, muscle, connective tissue, and vessels) excluding bone and cartilage. In addition, malignant tumors of peripheral nerve sheaths are usually included despite being ectodermal in origin. There are over 50 different histologic subtypes of STS with the most common being liposarcoma, leiomyosarcoma, fibrosarcoma, and synovial sarcoma. Malignant fibrous histiocytoma was historically the most common subtype but the majority of these are now classified as other subytpes including undifferentiated pleomorphic sarcomas. All suspected STS cases should be reviewed by a pathologist experienced in sarcomas given that about 10% of cases originally designated as STS are in fact not STS and about 20% are initially assigned the incorrect histologic subtype (5). While each histologic subtype may have certain specific clinical behaviors, all STS can generally be categorized into low-, intermediate-, and high-grade tumors. Low-grade tumors grow more slowly, can locally recur after resection, but have a low risk of distant metastases (about 5%). High-grade tumors tend to grow more rapidly, can recur locally, and have the added risk of distant metastasis that can approach 50% for large tumors greater than 5–10 cm in largest dimension.
The treatment of STS has advanced significantly over the past few decades. In particular, evidence has accumulated that in addition to surgery, there are important roles for radiation therapy and chemotherapy in the management of some STS patients. Optimal results from more conservative local treatment strategies require a multidisciplinary approach to the overall management of these patients. The team should include not only an experienced and specialized surgeon, but also a radiation oncologist, medical oncologist, pathologist, and diagnostic radiologist expert in the disease. Additional specialists who may be important in the care of these patients include plastic/reconstructive surgeons, physiatrists working with physical and occupational therapists, psychiatrists, psychologists, and social workers. For this relatively uncommon solid tumor that occurs ...