Rhabdomyosarcoma of the head and neck region: experience at the pediatric unit of the Istituto Nazionale Tumori, Milan.

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.