Biology of Ewing Sarcoma

Abstract

Sarcomas are a heterogeneous group of malignant tumors that are derived from mesenchymal tissues, including bone, muscle, and cartilage. In the last decade, we have gained significant new insights into the genetic abnormalities that underlie the pathogenesis of these tumors. Specific molecular alterations have been associated with specific histological subtypes of sarcomas, leading to a new classification of many sarcomas. Conventionally grouped in either soft-tissue or bone sarcomas according to the site of their origin, these tumors can now be genetically distinguished in two main groups: those carrying tumor-specific recurrent chromosome aberrations and those with complex karyotypes and variable genetic alterations [1, 2]. Sarcomas with recurrent molecular changes include, among others, Ewing sarcoma, synovial sarcoma, alveolar rhabdomyosarcoma, myxoid liposarcoma and myxoid chondrosarcoma.