ABSTRACT

Sarcomas are malignant tumours that arise from skeletal and extra-skeletal connective tissues, including the peripheral nervous system. In addition, malignant tumours of peripheral nerve sheaths are included despite being ectodermal in origin, as their clinical behaviour is similar to that of other sarcomas. Soft tissue sarcoma (STS) should be viewed at the molecular level as a disease of the involved genes. Involvement of genetic factors in the genesis of STS is manifest by the strong hereditary tendency for certain sarcomas. Sarcomas with recurrent chromosome translocations account for approximately one-third of all STSs. In contrast to the sarcomas that have recurrent, nonrandom chromosome translocations and relatively simple karyotypes, the molecular pathogenesis of sarcomas with non-specific genetic alterations and complex karyotypes has yet to be elucidated. Exposure to a few selected industrial chemicals including vinyl chloride, phenoxyacetic acid, arsenic, and phenoxy herbicides may be followed by the appearance of sarcomas.