Adults with Ewing's sarcoma primitive neuroectodermal tumor - Adverse effect of older age and primary extraosseous disease on outcome

Objective To assess outcome and prognostic factors for survival of adults with Ewing' s sarcoma/primitive neuroectodermal tumor (PNET). Background Ewing's sarcoma/PNET is a disease of childhood rarely seen in adults. Accor dingly, there is a relative paucity of published literature pertaining to o utcome for adults with this disease. Methods Between 1979 and 1996, 37 patients with newly diagnosed Ewing's sarcoma/PNE T were evaluated and treated at the Adult Sarcoma Program at Dana-Farber Ca ncer Institute and Brigham & Women's Hospital. Twenty-six patients had loca lized disease at presentation and II had metastatic disease. All but two pa tients received multiagent chemotherapy. Local treatment consisted of surge ry (7 patients), surgery and radiation therapy (19), radiation therapy (6), or no local treatment (5). Median follow-up for living patients was 100 mo nths (range 8 to 199). Results The 5-year survival rate for the group overall was 37% +/- 9%. The 5-year l ocal control rate was 85% +/- 7%. Significant favorable predictors for surv ival on univariate analysis included localized disease at presentation. pri mary origin in bone, primary size <8 cm, and a favorable objective response to chemotherapy. Patients with localized disease had a 5-year survival rat e of 49% +/- 11% compared with 0% for those with metastatic disease at pres entation. Multivariate analysis showed three significant independent predic tors for death: metastatic disease at presentation, primary origin in extra osseous tissue versus bone, and age 26 years or older. Conclusion Adult patients with Ewing's sarcoma/PNET at highest risk for death are thos e who are older than 26 years and have metastatic disease or an extraosseou s primary tumor. The development of novel therapies should target these hig hrisk groups.