Improved survival for children with parameningeal rhabdomyosarcoma: Results from the AIEOP soft tissue sarcoma committee

Autor: Milena Calderone, Gianni Bisogno, Costanza De Rossi, Alberto Donfrancesco, Giovanni Cecchetto, Modesto Carli, Sandro Dallorso, Andrea Ferrari, Yessika Gamboa, Guido Sotti, Lorenza Gandola, Angelo Rosolen
Rok vydání: 2008
Předmět:
Zdroj: Pediatric Blood & Cancer. 50:1154-1158
ISSN: 1545-5009
Popis: 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. Pediatr Blood Cancer 2008;50:1154–1158. © 2008 Wiley-Liss, Inc.
Databáze: OpenAIRE
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