Rhabdomyosarcoma of the oral and paraoral region

Oral rhabdomyosarcoma (RMS) was studied by analysis of eight such cases which presented over a 25-year period. Rhabdomyosarcoma was the fourth most common oral sarcoma (7.5%) from this period after osteosarcoma (32%), fibrosarcoma (19%), and chondrosarcoma (9%). In patients younger than 20 years, RMS was the second most common sarcoma (six cases) after osteosarcoma (ten cases). Combined analysis with 113 further cases documented in the literature showed the majority of cases (71.2%) were embryonal. The alveolar subtype was considered a distant second in frequency (12.3%) even though the pleomorphic subtype was apparently more common (16.4%). However, the pleomorphic cases were diagnosed before histologic criteria were established to discern this entity from other pleomorphic sarcomas and this data is probably unreliable. Site predilections were found for the soft palate, maxillary sinus and alveolus, posterior mandibular region, cheek and lip and possibly tongue. The gingiva and floor of mouth were uncommon sites. There was a predilection for occurrence in the first two decades with a decline in the third decade. Documentation of recent cases treated with a multidisciplinary approach indicated that lesions in the oral soft tissues have a good prognosis; 17 of 21 such cases showed no evidence of disease after a mean follow-up period of 7.2 years (SD = 4.4). In contrast, four of five cases in the posterior mandible resulted in death after a mean period of 1.1 years (SD = 0.3).