Rhabdomyosarcoma of the head and neck region: Experience at the Pediatric Unit of the Istituto Nazionale Tumori, Milan

Cristina Meazza, Andrea Ferrari, Michela Casanova, Maura Massimino, Roberto Luksch, Filippo Spreafico, Graziella Cefalo, Monica Terenziani, Daniela Polastri, Marta Podda, Serena Catania, Stefano Cereda, Lorenza Gandola, Franca Fossati-Bellani, Giulio Cantù

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: This study aims to describe the clinical features and outcome of pediatric patients with head and neck rhabdomyosarcoma (RMS), which can be divided into parameningeal, orbital, or nonorbital, nonparameningeal. Design: This is a retrospective single-institution analysis. Setting: The study was performed at the Istituto Nazionale Tumori, Milan, Italy. Methods: The study considered 142 consecutive patients <21 years treated from 1973 to 2003. Main Outcome Measures: Patients were treated using a multimodality approach: complete conservative surgery was performed at diagnosis in only two patients, all patients received chemotherapy, and most (91%) also had radiotherapy. Disease-free survival and overall survival were estimated according to the Kaplan-Meier method. Results: For the series as a whole, the 5-year disease-free and overall surival rates were 49% and 57%, respectively. The 5-year overall survival rate improved over the years from 38% before 1980 to 78% after 1990; it was 44% for parameningeal, 79% for orbital, and 77% for nonorbital, nonparameningeal RMS. Conclusions: The treatment of head and neck RMS is complex and necessarily multidisciplinary. Complete surgery is rarely feasible in the head and neck region. This study confirms that orbital RMS has a favourable outcome, whereas therapeutic results are generally unsatisfactory in parameningeal cases, although recent progress in radiotherapeutic techniques and in the efficacy of chemotherapy would suggest clear improvements in survival.

Original languageEnglish
Pages (from-to)53-59
Number of pages7
JournalJournal of Otolaryngology
Volume35
Issue number1
DOIs
Publication statusPublished - Feb 2006

Keywords

  • Head and neck rhabdomyosarcoma
  • Head and neck tumours
  • Orbital rhabdomyosarcoma
  • Parameningeal rhabdomyosarcoma
  • Pediatric tumours
  • Therapy

ASJC Scopus subject areas

  • Otorhinolaryngology

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