ArticlePloS one2025
Clinicopathological profile and survival in children with parameningeal rhabdomyosarcoma in resource-limited settings: A single-center experience from Uganda.
Article in PloS one, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundParameningeal rhabdomyosarcoma (PM-RMS) represents a diagnostic and therapeutic challenge, especially in low- and middle-income countries (LMICs), given its location and its propensity for local and intracranial extension. This study determined the clinical and pathological profile and survival in children with PM-RMS treated at a single tertiary cancer treatment facility in Uganda.
methodsThis was a retrospective study involving a review of records of children and adolescents aged below 18 years with rhabdomyosarcoma treated at the Uganda Cancer Institute (UCI) between January 2016 and December 2020. Kaplan-Meier survival analysis and Cox's proportional hazards model were used for five-year survival analysis.
resultsWe identified 32 PM-RMS cases with a median age of 4.8 years (range 1-15 years). The most common tumor sites were the infratemporal region (25.0%), middle ear (21.8%), and nasopharynx (18.8%). Most patients (90.6%) were IRS group III, and 34.4% had regional lymph node involvement. Embryonal and alveolar histologies represented 46.9% and 21.8%, respectively, with 31.3% unclassified. Nearly all patients (90.6%) received chemotherapy, but only 43.8% underwent radiotherapy for local control. One- and five-year overall survival rates were 65% and 12%, respectively. Regional nodal involvement and receipt of local control were the significant predictors of survival (adjusted HR 4.61 and 6.07, respectively).
conclusionOur study demonstrates a low survival rate among children with PM-RMS among the patient cohort, and treatment abandonment remains high. Regional nodal involvement and local control significantly predicted survival.
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