Sarcomas comprise a heterogeneous group of mesenchymal neoplasms. They can be grouped into 3 general categories, soft tissue sarcoma, visceral and primary bone sarcoma, which have different staging and treatment approaches. Soft tissue sarcomas are typically classified on the basis of genetic alterations and light-microscopic examination of hematoxylin-eosin-stained tissue, in which recognizable morphological characteristics of normal tissues are identified. Sarcomas are further characterized by histologic grade. The 3 most important prognostic variables are grade, size, and location of the primary tumor. This review includes a discussion of both soft tissue sarcomas (unclassified sarcoma, liposarcoma, leiomyosarcoma, synovial sarcoma, dermatofibrosarcoma protuberans, angiosarcoma, Kaposi sarcoma, gastrointestinal stromal tumor, rhabdomyosarcoma, ...) and primary bone sarcomas (osteosarcoma, Ewing sarcoma and chondrosarcoma). The approach to a patient with a sarcoma begins with a biopsy that obtains adequate tissue for diagnosis without interfering with subsequent optimal definitive surgery. Subsequent treatment depends on the specific type of sarcoma. Due to the absence of clear knowledge for incidence rate, we conducted in 2005 and 2006 an exhaustive analysis of all diagnosed cases in the Rhône-Alpes region. Because sarcomas are relatively uncommon yet comprise a wide variety of different entities, second opinion was systematically performed for all included cases.