ABSTRACT
Sarcomas comprise a group of relatively rare, anatomically and histologically diverse neoplasms. These tumors share a common embryologic origin, arising primarily from mesenchymal tissue. The notable exceptions are malignant peripheral nerve sheath tumors and primitive neuroectodermal tumors, also known as Ewing sarcomas, which are believed to arise from neuroectodermal tissue. Despite the fact that the somatic soft tissues account for as much as 75% of total body weight, neoplasms of the soft tissues are comparatively rare, accounting for less than 1% of adult malignancies and 15% of pediatric malignancies. The annual incidence of soft tissue sarcomas in the United States is about 15,000 if those arising in organs are included, according to SEER estimates. For bone sarcomas, the incidence is about 2500 cases. For sarcomas arising in somatic soft tissue, there are only about 8000 new cases, but only about 4% of these or about 300 cases arise in the head and neck. Nonetheless, the overall mortality rate of sarcomas is almost 50% at all sites, and tumors of the skull base are particularly hard to cure due to the inability to perform adequate oncologic surgery due to anatomic constraints.
