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Background: Rhabdomyosarcoma (RMS) is an aggressive malignant neoplasm of mesenchymal origin. Its common sites of occurrence are the extremities and the genitourinary system, being less frequently seen in the orofacial region. There is paucity of literature, especially from sub-Saharan Africa on orofacial RMS. Materials and Methods: Records of all patients managed for orofacial RMS between January 2013 and November 2017 at the Department of Dental and Maxillofacial surgery of Usmanu Danfodiyo University Teaching Hospital were obtained. Data retrieved include sociodemographics, clinical features (including the tumor, node, and metastasis [TNM] stage and Intergroup RMS Study risk classification), histological type, and mode of treatment. Data were recorded and analyzed using IBM SPSS Statistics for Windows version 20 (Armonk, IBM Corp., NY, USA). Results: Twenty-one patients were included. There were 17 (81.0%) males and 4 (19.1%) females, giving a male-to-female ratio of 4.3:1. Age ranged from 2 to 76 years (mean [±standard deviation] of 15.8 [±16.8]). Majority of them belonged to the low socioeconomic group. The duration of the symptoms ranged from 2 to 9 months. The midface was the most commonly involved sites. Majority of the cases were nonparameningeal 14 (66.7%). Regional nodal metastasis was clinically present in 9 (42.9%) cases. Embryonal RMS was the most frequently encountered histological type (11 [52.4%]). Treatment was multimodal in 11 (52.4%) cases and 3 (14.1%) had recurrence. Conclusion: Orofacial RMS may occur in any age group although it may be more commonly seen in the first decade of life. Unimodal treatment may be successfully deployed in carefully selected cases. Prognosis remains poor in our climes.
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