ESTRO-SIOPE guideline: Clinical management of radiotherapy in atypical teratoid/rhabdoid tumors (AT/RTs)

Radiother Oncol. 2024 Jul:196:110227. doi: 10.1016/j.radonc.2024.110227. Epub 2024 Mar 14.


Background and purpose: Treatment of patients with atypical teratoid/rhabdoid (AT/RT) is challenging, especially when very young (below the age of three years). Radiotherapy (RT) is part of a complex trimodality therapy. The purpose of this guideline is to provide appropriate recommendations for RT in the clinical management of patients not enrolled in clinical trials.

Materials and methods: Nine European experts were nominated to form a European Society for Radiotherapy and Oncology (ESTRO) guideline committee. A systematic literature search was conducted in PubMed/MEDLINE and Web of Science. They discussed and analyzed the evidence concerning the role of RT in the clinical management of AT/RT.

Results: Recommendations on diagnostic imaging, therapeutic principles, RT considerations regarding timing, dose, techniques, target volume definitions, dose constraints of radiation-sensitive organs at risk, concomitant chemotherapy, and follow-up were considered. Treating children with AT/RT within the framework of prospective trials or prospective registries is of utmost importance.

Conclusion: The present guideline summarizes the evidence and clinical-based recommendations for RT in patients with AT/RT. Prospective clinical trials and international, large registries evaluating modern treatment approaches will contribute to a better understanding of the best treatment for these children in future.

Keywords: Atypical teratoid rhabdoid tumor; Brain tumor; Children; Radiotherapy.

Publication types

  • Practice Guideline

MeSH terms

  • Child, Preschool
  • Humans
  • Infant
  • Radiotherapy Dosage
  • Rhabdoid Tumor* / radiotherapy
  • Rhabdoid Tumor* / therapy
  • Teratoma* / radiotherapy

Supplementary concepts

  • Teratoid Tumor, Atypical