Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma

Objective To assess outcome and identify predictors of survival of adults with rhabdo myosarcoma. Summary Background Data The literature on adult rhabdomyosarcoma is limited. Few studies have ident ified predictors of long-term survival in this patient population. Methods Thirty-nine adults with rhabdomyosarcoma were treated between 1973 and 1996 and prospectively followed. Outcomes were assessed with respect to patient and tumor characteristics, local treatment, and response to chemotherapy. Results Twenty-six patients had localized/locoregional disease and 13 patients had metastatic disease at presentation. Twenty-one patients underwent attempted curative resection, 27 received radiotherapy, and 37 received chemotherapy . Median follow-up for surviving patients was 152 months. The overall 5- an d 10-year survival rates were 31 % and 27%, respectively. Five-year surviva l rates for patients with tumors less than 5 cm, 5 to 10 cm, and more than 10 cm were 60%, 14%, and 0%, respectively. Patients with localized/locoregi onal disease at presentation had a 44% 5-year survival rate; there were no 5-year survivors among patients with metastatic disease. Patients who had a complete response to chemotherapy had a 5-year survival rate of 57%, compa red with a rate of only 7% for poor responders. Metastatic disease at prese ntation and poor response to chemotherapy were independent predictors of de ath on multivariate analysis. Conclusions Age, location, nodal status, and histologic subtype do not appear be associ ated with survival in adults with rhabdomyosarcoma treated with multimodal therapy. Metastatic disease at presentation and poor response to chemothera py are strongly associated with poor prognosis. Future systemic therapies s hould be targeted to patients with localized/locoregional disease and parti al responders to conventional chemotherapy.