Predictors of survival among children and adolescents with rhabdomyosarcoma treated in a single resource-limited centre -Uganda.
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BACKGROUND: The treatment outcomes for children and adolescents with rhabdomyosarcoma (RMS) in low-income countries are poor. However, there is a paucity of literature on RMS and its management outcomes in low-resource settings. We evaluated the treatment of RMS with the aim of identifying prognostic factors during management to improve outcomes. METHODS: We sourced data on children under 18 years treated for rhabdomyosarcoma at the Uganda Cancer Institute between January 2016 and December 2020. Kaplan-Meier survival analysis and Cox's proportional hazards model were used for five-year survival analysis. RESULTS: One hundred twenty-eight RMS cases were identified, with a median age of 6.0 years (IQR 3.6-10.0). The most common primary tumour site was the head and neck region, comprising non-parameingeal sites, 37 (28.9%); parameingeal sites, 32 (25.0%); and orbital tumours, 17 (13.3%). Overall, 68 (53.1%) of the primary tumour sites were unfavourable sites. Seventeen (13.3%) patients had metastatic disease at diagnosis, primarily to the lungs, 11 (64.8%). Embryonal and alveolar RMS accounted for 50.0% and 20.3% of the cases, respectively. Only 31 (24.2%) of the patients underwent surgery, and 36 (28.1%) were irradiated. The treatment completion rate was 33.6%, while 46.1% abandoned treatment. Only 25 (19.5%) patients were alive at the time of the study, 65 (50.8%) had died, and 38 (29.7%) had an unknown status. The five-year overall and event-free survival rates were 35% and 30%, respectively. Orbital primary tumour site (HR = 2.86; 95% CI 1.12-7.31; p = 0.028), metastatic disease (HR = 4.09; 95% CI 2.01-8.31; p < 0.001), elevated serum lactate dehydrogenase at diagnosis above 400 U/L (HR = 2.80; 95% CI 1.46-5.33; p = 0.002), and lack of local control (HR = 3.33; 95% CI 1.34-8.29; p = 0.010) were significant factors for poor survival. CONCLUSION: Rhabdomyosarcoma outcomes in Ugandan children are largely poor, with high treatment abandonment and mortality. Concerted, multidisciplinary efforts are needed to improve outcomes in this setting.