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

Gianni Bisogno, Costanza De Rossi, Yéssika Gamboa, Guido Sotti, Andrea Ferrari, Sandro Dallorso, Alberto Donfrancesco, Giovanni Cecchetto, Milena Calderone, Lorenza Gandola, Angelo Rosolen, Modesto Carli

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)1154-1158
Number of pages5
JournalPediatric Blood and Cancer
Volume50
Issue number6
DOIs
Publication statusPublished - Jun 2008

Keywords

  • Childhood soft tissue sarcoma
  • Hyperfractionated and accelerated radiotherapy
  • Parameningeal rhabdomyosarcoma

ASJC Scopus subject areas

  • Cancer Research
  • Pediatrics, Perinatology, and Child Health
  • Hematology

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