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Is There a Place for Surgical Management?

Hamid Daya, MD, FRCS(ORL); Helen S. L. Chan, MBBS, FRCPC; Wilma Sirkin, MBBCh, FRCPC; Vito Forte, MD, FRCSC

Arch Otolaryngol Head Neck Surg. 2000;126:468-472.

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.

From the Departments of Pediatric Otolaryngology (Drs Daya and Forte) and Pathology (Dr Sirkin), and the Division of Hematology/Oncology, Department of Pediatrics (Dr Chan), The Hospital for Sick Children, Toronto, Ontario.