Pediatric rhabdomyosarcoma of the head and neck - Is there a place for surgical management?

Objective: To review and evaluate the place of surgical treatment in the ma nagement of rhabdomyosarcoma of the head and neck in children. Design: Retrospective analysis of patient charts from January 1, 1972, to D ecember 31, 1998. Setting: Tertiary pediatric referral center. Patients: Twenty-nine consecutive children with non-orbital head and neck r habdomyosarcoma. Interventions: Surgery, chemotherapy, and radiotherapy. Main Outcome Measures: Disease-free survival and long-term morbidity from t reatment. Results: Twenty patients had parameningeal and 9 had nonparameningeal head and neck tumors. All were treated with chemotherapy. For 18 patients, diagn ostic biopsies were performed and they received radiotherapy. Eleven patien ts underwent surgery as definitive therapy. Using the Intergroup Rhabdomyos arcoma Study (IRS) staging system, 5 of these 11 patients had complete rese ction of tumor (IRS group I) and avoided radiotherapy. The other 6 patients required radiation because of compromised, regional, or incomplete resecti on of tumor. One had undergone regional resection with nodal involvement, a nd 2 had compromised resections with microscopic residual disease (IRS grou p II). Three had incomplete resections with gross residual, tumor (IRS grou p III). Only 1 patient who underwent surgery ultimately died from recurrenc e at 2.7 years after an incomplete resection. The other 10 patients were re lapse free at a median follow-up of 3.7 years (range 0.8-21.0 years). Long- term surgical morbidity was seen in 36% (4/11) of the patients and included facial nerve paralysis, trismus, and cosmetic deformity. Conclusions: Children with localized disease of the head and neck are able to undergo complete surgical resection, with low long-term surgical morbidi ty. By undergoing complete surgical resection, these children are able to a void radiotherapy and its long-term complications, with no compromise in su rvival.