Rhabdomyosarcoma of the maxillofacial region in children and adolescents: Report of 9 cases and literature review

Objective: To review clinical presentation, histology, treatment and survival for pediatric maxillofacial rhabdomyosarcoma (RMS) and evaluate the role of surgical treatment.

Study design: Retrospective analysis of medical charts.

Methods: Files of patients, treated for primary maxillofacial RMS from 1997 to 2016, were examined for clinical presentation, staging, histology, treatment protocol and complications, recurrence and final outcome.

Results: Our cohort included 4 male and 5 female patients (mean age 8.47 years). One tumor, occupying the infratemporal space, was parameningeal; the other 8, located at the mandible (4) or the maxilla/zygomatic bone (4) were non-parameningeal. All patients received chemotherapy preoperatively. Surgery was performed in 7 patients, of whom 4 received postoperative radiotherapy. The histological type was alveolar (5) or embryonal (4). Overall survival hitherto was 66,6%, depending on histology (40% and 100% for the alveolar and embryonal type respectively).

Conclusions: Pediatric maxillofacial RMS originated mostly from the facial skeleton and most tumors were non-parameningeal. The alveolar type was slightly more common. An individualized multidisciplinary approach combining chemotherapy and local control mostly with surgery and selectively with radiotherapy has proven successful for the treatment of non-orbital, non-parameningeal maxillofacial RMS. Histology was a major treatment determinant and the most important prognostic factor.