Treatment Results of Pediatric Nonorbital Head and Neck Rhabdomyosarcoma and Related Prognostic Factors / 대한이비인후과학회지

ABSTRACT

BACKGROUND AND OBJECTIVES: The survival rate for rhabdomyosarcoma has been improved much after introduction of multimodality treatment. We reviewed the treatment results and investigated the related prognostic factors of pediatric nonorbital head and neck rhabdomyosarcoma. MATERIALS AND METHOD: Medical records of 27 children treated for nonorbital head and neck rhabdomyosarcoma from January 1986 to December 2000 were analyzed. Patients' ages at diagnosis ranged from 1 to 169 months (mean 75 months). The pathologic subtypes were predominantly embryonal with 23 patients (85%), followed by alveolar subtype with 1 (4%), and uncertain one with 3 (11%). RESULTS: The overall 5-year survival rate was 69%. Of 27 patients, 20 had parameningeal and 7 had nonparameningeal tumors. Eleven (41%) had cranial nerve involvement with frequent abducens nerve and facial nerve involvement. Ten (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 an important prognostic factor. There is no indication of initial aggressive surgical resection for the tumors located in the parameningeal site, which is difficult to access.