Prevalence, Risk Factors and Management of Ocular Rhabdomyosarcoma (ORMS) in Africa: A Systematic Review

Abstract

Rhabdomyosarcoma (RMS) is the most common paediatric orbital malignancy, accounting for about 10% of orbital tumours. Reported survival varies widely, ranging from as low as 10% with unimodal treatment to over 70% with multimodal therapy. Data from Africa remain sparse, with studies showing wide variations in methodology and treatment protocols. This systematic review and meta-analysis aimed to determine the prevalence, risk factors, and treatment modalities for ocular rhabdomyosarcoma in Africa. Following PRISMA guidelines and using a PICO framework, relevant databases were searched with appropriate keywords and medical subject headings. Thirty-nine studies met the inclusion criteria and were analysed using Microsoft Excel 365 and Stata IC software. The findings revealed that prevalence, incidence, and risk factors could not be reliably determined due to heterogeneity and inconsistencies in study designs. Evaluation of treatment outcomes was also not feasible as most studies lacked adherence to standardized guidelines, limiting comparability. Nevertheless, proptosis emerged as the most frequent presenting symptom, appearing earlier in the disease course than in other ocular malignancies. Surgery and chemotherapy were the most commonly employed treatment modalities. Overall, the evidence from Africa is inconclusive, and the variability in study designs coupled with the absence of standardized reporting reduces the generalizability of findings. Strengthening oncology care in Africa requires standardization of research protocols, subspecialty training, and the establishment of multidisciplinary oncology teams at all levels.