Background: Ewing's sarcoma/primitive neuroectodermal tumours (ES/PNET) are aggressive musculoskeletal tumours with a predilection for young people. With current treatments, significant numbers of patients relapse and survival is poor for those with metastatic disease.
Objective: To review current experimental treatment strategies in ES/PNET and prospects for the future.
Methods: A review of the literature and recent meeting presentations on established and experimental cytotoxic and biological therapies in the treatment of ES/PNET was performed.
Results/conclusion: New combinations of conventional and emerging cytotoxics show some promise. Molecular techniques are being used to identify high-risk patients and potential cellular targets. Several novel biologically targeted agents have demonstrated encouraging preliminary clinical efficacy; it is hoped these combined with current chemotherapeutic agents may improve outcome in ES/PNET.