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Introduction <p>Extracranial 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.</p> Materials and methods <p>Retrospective analyses of children (≤ 15&#xa0;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&#xa0;weeks (surgery and/or radiotherapy).</p> Results <p>Ninety-six children with eMRT were registered during this period, with a median age of 18.5&#xa0;months (IQR, 0.7–176&#xa0;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&#xa0;months (range, 2–64&#xa0;months), the 2-year EFS and OS of the treated patients (<i>n</i> = 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 (<i>p</i> = 0.02).</p> Conclusion <p>Extracranial 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.</p>

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Treatment of extracranial malignant rhabdoid tumors in children with surgery and or radiotherapy on a multiagent chemotherapy protocol- insights from a single centre cohort

  • 期刊论文

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  • 发表日期：

    2026年5月27日

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  • Vignesh Subramani,
  • Badira Cheriyalinkal Parambil,
  • Maya Prasad,
  • Venkata Ram Mohan Gollamudi,
  • Sajid Qureshi,
  • Siddhartha Laskar,
  • Nehal Khanna,
  • Jifmi Jose,
  • Mukta Ramadwar,
  • Poonam Panjwani,
  • Vasundhara Smriti,
  • Akshay Baheti,
  • Sneha Shah,
  • Girish Chinnaswamy

摘要

Introduction

Extracranial 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 methods

Retrospective 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).

Results

Ninety-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).

Conclusion

Extracranial 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.