ArticleBMC cancer2026
Treatment of extracranial malignant rhabdoid tumors in children with surgery and or radiotherapy on a multiagent chemotherapy protocol- insights from a single centre cohort.
Article in BMC cancer, 2026. 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
introductionExtracranial malignant rhabdoid tumors (eMRTs) are rare childhood cancers with poor outcomes and no optimal therapeutic strategies. We report our experience in children with eMRT treated at a tertiary hospital in India. MATERIALS AND
methodsRetrospective analyses of children (≤ 15 years) with pathologically confirmed eMRT, treated between January 2011 to June 2023, were performed. The staging was based on whole-body 18F-FDG PET/CT and brain MRI. Children treated with a curative intent received a multimodality protocol consisting of chemotherapy and local treatment at 10-12 weeks (surgery and/or radiotherapy).
resultsNinety-six children with eMRT were registered during this period, with a median age of 18.5 months (IQR, 0.7-176 months). Thirty patients (31%) had renal MRT, and 66 (69%) had extrarenal MRT. Among the 73 patients with metastatic disease, only two patients proceeded with curative therapy after discussion with their families. At a median follow-up of 12 months (range, 2-64 months), the 2-year EFS and OS of the treated patients (n = 25, Stage I/II-12, III/IV-13) were 37.1% (SE ± 10%) and 45% (SE ± 11.2%), respectively. The application of surgery and radiotherapy alone had prognostic significance for EFS and OS, with the best outcomes for those who received both (p = 0.02).
conclusionExtracranial MRT is a highly aggressive tumor in young children with very poor outcomes. Incorporating both surgical resection and radiotherapy into local treatment, where feasible, may improve patient outcomes.
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