Ewing's sarcoma and primitive neuroectodermal tumor of hand and forearm

Purpose: Because Ewing's sarcoma (EWS) is extremely rare in the hand and forearm, only limited data are available for planning treatment or predicting prognosis. Methods: Data of 33 patients with EWS of the forearm or hand who were enrolled in studies of the German Association for Paediatric Oncology/Haematology and the European Intergroup Cooperative Ewing's Sarcoma Study Group were analyzed. Patients received neoadjuvant multi-agent chemotherapy according to the valid protocol. Local treatment consisted of surgery (n=7), radiotherapy (n=7), or a combination of both (n=19). Results: The 5/10-year overall survival rate was 84.1% (95% CI: 71.2-96.9)/74.1% ( 95% CI: 56.8-91.5), and both 5/10-year event-free survival rate were 71.3% ( 95% CI: 55.4-87.1). Only one of seven patients with secondary metastases was in remission at the time of analysis. One patient with local recurrence and another with primary metastases died. Altogether, eight of 33 patients died of their disease. The event-free survival rate was 80.6% in patients with good response to chemotherapy and 33.3% in patients with poor response. Surgery in combination with radiotherapy achieved a higher survival rate compared with radiotherapy or surgery alone. One out of two patients with nonwide margins of resection died of disease. Conclusions: The results demonstrate a remarkably high survival rate for patients with EWS of the hand and forearm.