Published in PLOS One on 2025-10-09, this study is presented as an attributed evidence briefing. The excerpts below are the original researchers’ words, reproduced under their Creative Commons Attribution licence. Health Signal has selected the abstract and, where needed, opening background paragraphs; the complete methods, results, limitations and references remain in the linked paper.
Original authors: Richard Nyeko, Fadhil Geriga, Racheal Angom, Joyce Balagadde Kambugu, Jaques van Heerden
Source: PLOS One • DOI 10.1371/journal.pone.0334140
Read the complete primary study ↗ · Creative Commons Attribution licence
From the researchers
Abstract
Parameningeal 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.
This 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.
We 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).
Our 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.
Background
Parameningeal rhabdomyosarcoma (PM-RMS) is a sub-classification of rhabdomyosarcoma (RMS) with a distinct pathophysiology and poor prognosis [1,2]. PM-RMS encompasses tumors arising from the parameningeal region, including the nasopharynx, nasal cavity, parapharyngeal space, paranasal sinuses, infratemporal and pterygopalatine fossa, middle ear, and mastoid, or from outside this region but with parameningeal/intracranial extension [2,3]. Twenty percent of RMS occurs in the parameningeal area [3].
Treatment of PM-RMS, just as for RMS in general, relies on the use of a multimodal therapeutic approach involving a combination of multi-agent therapy combined with effective local control using surgery (primary or secondary) and radiotherapy (RT) [4–6]. Local control of the primary tumor, which can arise at many distinct anatomic sites, as well as control of disseminated disease, remains the hallmark of curative therapy in RMS in general [6].
However, parameningeal sites of RMS have been shown to portend an unfavorable prognosis compared to other sites of origin [1,3,7]. The complex anatomy and proximity to critical anatomic structures make surgical exploration and complete resection a therapeutic challenge, which precludes local control in the majority of cases. Additionally, limited or delayed radiotherapy (RT) in young children decreases treatment outcomes, and dissemination is increased via the cerebrospinal fluid (CSF) [2,8]. The presence of intracranial extension and cranial nerve palsies at diagnosis has been shown to herald a poor prognosis with the risk of developing local and distant recurrence [9,10].
Reading this evidence
Use the full paper to check the study design, participant selection, comparator and outcome definitions before drawing a practical conclusion. An association does not by itself show cause and effect. A reported performance measure needs its denominator and uncertainty. A finding from a particular facility, sample or time period should retain that scope when it is discussed elsewhere.
This briefing makes the source accessible within the wider technology & industry desk. It does not assess an individual patient or certify a product. When considering relevance to another health system, examine the original authors’ account of context and limitations, then seek local implementation evidence. The related reporting below provides other studies to read alongside this one; it is a topic connection, rather than a claim that their results are directly comparable.
© 2025 Richard Nyeko, Fadhil Geriga, Racheal Angom, Joyce Balagadde Kambugu, Jaques van Heerden. Excerpts reproduced under CC BY. Selection, formatting and contextual notes by Health Signal. The researchers are the authors of the source study, not members of Health Signal’s staff.
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