Surgical management of craniofacial and skull base rhabdomyosarcomas

Craniofacial and skull base rhabdomyosarcomas (RMSs) are rare. Surgeons are involved both in the resection and the reconstruction of these tumors and in the management of late sequelae of earlier treatment. These tumors are highly heterogeneous, and the initial presentation may be insidious. We wished to determine how diagnostic difficulties could be overcome and how management outcomes could be optimized.

All cases of RMS in patients who presented to our craniofacial program between 2004 and 2008 were retrospectively reviewed.

Eight consecutive patients with RMS were treated. Six patients are Maori. Four patients had surgical resections of their primary tumors including 3 with curative intent. One had a recurrent tumor, and 3 had functional reconstruction after an earlier treatment. Inaccuracies in histologic diagnosis occurred in 2 patients. Two patients developed postoperative cerebrospinal fluid leakage. Two patients had leptomeningeal involvement and died within 10 months. All patients treated with curative intent achieved complete macroscopic margins. Two remained disease-free at 10 and 22 months.

Treatment must be in the context of a multidisciplinary team with the appropriate craniofacial, neurosurgical, and reconstructive skills. Expedient and accurate histologic diagnoses by experienced pathologists are critical in optimizing the outcomes. Oncologists must be well resourced to administer chemoradiation in a timely manner. Leptomeningeal invasion is a dire prognostic feature. Cerebrospinal fluid leak after resection must be identified early and repaired. Late consequences of early childhood craniofacial cancer treatment may be ameliorated by tailored reconstructions. The predominance of Maori in our series has not been reported previously.