Abstract

Objectives: We reviewed the treatment results and investigated the related prognostic factors of pediatric nonorbital head and neck rhabdomyosarcoma. Methods: Medical records of 27 children with nonorbital head and neck rhabdomyosarcoma from January 1986 to December 2000 were analyzed. Patient ages at diagnosis ranged from 1 to 169 months (mean, 75 months). The pathologic subtypes were predominantly embryonal in 23 patients (85%), alveolar in 1(4%), and uncertain in 3(11%). Results: Overall 5-year survival was 69%. Of 27 patients, 20 had parameningeal and 7 had nonparameningeal tumors; 11(41%) had cranial nerve involvement with frequently abducens nerve and facial nerve involvement; 10(37%) had skull base or brain invasion. The 5-year survival of patients with skull base or brain invasion was 40% and without skull base or brain invasion was 88%. Parameningeal site (68% vs 71%) and cranial nerve involvement (64% vs 73%) were not significant prognostic factors. Conclusion: Skull base or brain invasion was important prognostic factor. There is no need of initial aggressive surgical resection for the tumors located in parameningeal site which is difficult to access.

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