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Title: Improved survival for children with parameningeal rhabdomyosarcoma: results from the AIEOP soft tissue sarcoma committee. Author: Bisogno G, De Rossi C, Gamboa Y, Sotti G, Ferrari A, Dallorso S, Donfrancesco A, Cecchetto G, Calderone M, Gandola L, Rosolen A, Carli M. Journal: Pediatr Blood Cancer; 2008 Jun; 50(6):1154-8. PubMed ID: 18300319. Abstract: BACKGROUND: Parameningeal rhabdomyosarcoma (PM-RMS) is a rare, highly malignant pediatric tumor arising from locations adjacent to the meninges, from where it can spread intracranially. PROCEDURE: We reviewed 109 children with non-metastatic PM-RMS enrolled in the Italian RMS79, RMS88 and RMS96 protocols over a 24-year period. All patients received intensive chemotherapy and standard or hyperfractionated and accelerated radiotherapy. Some had delayed surgery. RESULTS: Five-year overall survival rose from 40% in the RMS79 to 72% in the RMS88 and RMS96 protocols (P = 0.01), where more intensive chemotherapy and hyperfractionated accelerated radiotherapy (HART) was used. Delayed surgery after initial treatment was statistically associated with a better prognosis. Unfavorable tumor characteristics for RMS arising in other sites, for example, histology, invasiveness or node involvement, did not predict outcome for PM-RMS. CONCLUSION: Outcome in PM-RMS patients enrolled in three consecutive Italian protocols has progressively improved, as a result of intensive chemotherapy, delayed surgery and, possibly, HART, though improved imaging and radiotherapeutic tools may have had a role as well.[Abstract] [Full Text] [Related] [New Search]