TY - JOUR
T1 - Improved survival for children with parameningeal rhabdomyosarcoma
T2 - Results from the AIEOP soft tissue sarcoma committee
AU - Bisogno, Gianni
AU - De Rossi, Costanza
AU - Gamboa, Yéssika
AU - Sotti, Guido
AU - Ferrari, Andrea
AU - Dallorso, Sandro
AU - Donfrancesco, Alberto
AU - Cecchetto, Giovanni
AU - Calderone, Milena
AU - Gandola, Lorenza
AU - Rosolen, Angelo
AU - Carli, Modesto
PY - 2008/6
Y1 - 2008/6
N2 - 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.
AB - 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.
KW - Childhood soft tissue sarcoma
KW - Hyperfractionated and accelerated radiotherapy
KW - Parameningeal rhabdomyosarcoma
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U2 - 10.1002/pbc.21527
DO - 10.1002/pbc.21527
M3 - Article
C2 - 18300319
AN - SCOPUS:42349110115
VL - 50
SP - 1154
EP - 1158
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
SN - 1545-5009
IS - 6
ER -