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Improved survival for children with parameningeal rhabdomyosarcoma: Results from the AIEOP soft tissue sarcoma committee
Author(s) -
Bisogno Gianni,
De Rossi Costanza,
Gamboa Yéssika,
Sotti Guido,
Ferrari Andrea,
Dallorso Sandro,
Donfrancesco Alberto,
Cecchetto Giovanni,
Calderone Milena,
Gandola Lorenza,
Rosolen Angelo,
Carli Modesto
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21527
Subject(s) - medicine , rhabdomyosarcoma , radiation therapy , chemotherapy , soft tissue sarcoma , sarcoma , surgery , radiology , soft tissue , pathology
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.