Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm consisting of cells derived from the primitive mesenchyme that exhibit a profound tendency to myogenesis. About 35% of RMS arises in the head and neck, being classified as parameningeal and non-orbital non-parameningeal forms. Parameningeal tumors carry the worst prognosis. The use of contemporary, multi-agent chemotherapy, radiotherapy, and surgery has made treatment of the disseminated disease possible, and has significantly improved overall survival from 25% in 1970 to 70% in 1991. Here, we present the management of two cases of orofacial RMS in adolescents: an 18-year-old, white female that had a 9-month history of a nodule in the left buccal mucosa, and a 19-year-old, white male who had been aware of a nodule in the left, posterior maxillary ridge with progressive growth for 4 months. Before final diagnosis, both cases were previously treated as inflammatory lesions. Their clinicopathological aspects, treatment, and poor survival as a consequence of delays in diagnosis are discussed.
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