Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm, with varying degrees of striated muscle cell differentiation and a relative predilection for the head and neck region. The objectives of this study were to determine the clinical and histological patterns of RMS, to assess the sociodemographic profile of reported RMS cases, and to highlight the management challenges and outcomes of these cases in a country lacking resources such as Nigeria. Patients diagnosed with RMS, based on clinical and histological evaluation, and recorded in the cancer registry of two maxillofacial centres in Nigeria were reviewed. 21 patients met the inclusion criteria for this study. Their ages ranged from 24 days to 42 years. In all of them multiple anatomical sites were involved. The primary site of the lesion could not be determined. Embryonal and alveolar subtypes were mainly found in the maxilla. There was no statistically significant association between the site of the lesion and the histological type (p=0.39). The patients' age was statistically significantly associated with the histological type (p=0.008). Most patients reported at an advanced stage, contributing to the poor prognosis and management outcome of cases evaluated.
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