Models for Sarcomas

Abstract

Sarcomas are rare tumours in patients under the age of 20. Because of their characteristic chromosomal translocations Ewing's sarcomas have been a paradigm for understanding the biology of sarcomas. Since the survival rates in Ewing's sarcoma patients with metastasis and relapse are still unsatisfactory, new therapeutic options have to be developed. Model systems based on fibroblast cell lines have helped in understanding the biology of the transforming protein EWS/FLI1. Cell lines established from primary Ewing's sarcoma are now in use to test new therapeutic options. Since these models only poorly reflect the conditions within the patients, xenograft mouse models have recently been developed to study the biology of Ewing's sarcoma in a more relevant in vivo environment. These new model systems will help define novel therapeutic targets and support the preclinical evaluation of new treatments.