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 management of rhabdomyosarcoma of the head and neck in children.

DESIGN

Retrospective analysis of patient charts from January 1, 1972, to December 31, 1998.

SETTING

Tertiary pediatric referral center.

PATIENTS

Twenty-nine consecutive children with nonorbital head and neck rhabdomyosarcoma.

INTERVENTIONS

Surgery, chemotherapy, and radiotherapy.

MAIN OUTCOME MEASURES

Disease-free survival and long-term morbidity from treatment.

RESULTS

Twenty patients had parameningeal and 9 had nonparameningeal head and neck tumors. All were treated with chemotherapy. For 18 patients, diagnostic biopsies were performed and they received radiotherapy. Eleven patients underwent surgery as definitive therapy. Using the Intergroup Rhabdomyosarcoma Study (IRS) staging system, 5 of these 11 patients had complete resection of tumor (IRS group I) and avoided radiotherapy. The other 6 patients required radiation because of compromised, regional, or incomplete resection of tumor. One had undergone regional resection with nodal involvement, and 2 had compromised resections with microscopic residual disease (IRS group II). Three had incomplete resections with gross residual tumor (IRS group III). Only 1 patient who underwent surgery ultimately died from recurrence at 2.7 years after an incomplete resection. The other 10 patients were relapse 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 morbidity. By undergoing complete surgical resection, these children are able to avoid radiotherapy and its long-term complications, with no compromise in survival.