Purpose. We analyzed the outcome of 47 patients with superficial facial rhabdomyosarcoma (RMS) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols-III, -IV-Pilot, and -IV. Methods. We reviewed patients' records. Clinico-pathologic features, treatment, and outcome were examined to identify prognostic factors. Results. Thirty-two patients were males; 35 patients were 1-9 years old at diagnosis. Tumor sites were buccal/cheek (N = 21), external nasal/nasolabial (N = 12), lip/chin (N = 9), and masseter (N = 5). Patients (46/47) had localized disease: 18 biopsy only (Group III), 17 microscopic residual tumor (Group II), and 11 complete resection without residual tumor (Group I). Eight-year estimated event-free survival (EFS) and overall survival (OAS) rates were 61% and 65%. Patients <12 months old had inferior EFS, 21%, compared to ∼68% in older patients (P = 0.077). Eight-year EFS rates were 80% for females and 50% for males (P = 0.096). Eight-year EFS rates were 72% in 33 patients without regional lymph-nodal tumor and 39% in 14 patients with regional nodal tumor (P = 0.07). Eight-year EFS rates were 72% for 22 patients with embryonal RMS and 53% for 23 patients with alveolar RMS (P = 0.28). Location of the primary tumor was not significantly related to outcome. Conclusions. Patients with superficial facial RMS often have localized, grossly resectable lesions at the time of presentation. Favorable prognostic factors include age >12 months, female gender, embryonal histology, and no lymph-nodal tumor.
- Childhood/adolescent superficial facial rhabdomyosarcoma
- IRSG protocols
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health