Tumeurs PNET/Ewing : prise en charge actuelle et perspectives

Abstract

Ewing tumours are characterised as tumours consisting of small, blue, round malignant cells that may exhibit varying degrees of neural differentiation. Most of them arise in bony sites, and they represent the second commonest primary osseous malignancy in and adolescence and young adults. During the past 30 years, chemotherapy has increased survival from less than 5% to 65–70% in localized tumours and to 25–30% in primary metastatic tumours. Surgery is a major tool, whereas advances in imaging techniques have improved treatment indication and optimization. Radiotherapy remains useful, either alone or in addition to surgery, and new techniques (conformational RT and IMRT) will reduce short-term toxic effects. However, long-term toxic effects are also of major concern. Clinical and biological prognostic factors has been clearly identified and should guide the therapeutic choice for these patients. The metastatic Ewing tumours are of extremely poor prognosis, and impose the development of new therapeutic agents. This article is a review of the data available in 2009 concerning Ewing's sarcoma either as biologic aspects or as therapeutic aspects.