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<p>Paediatric kidney tumours are generally associated with a favourable survival rate. Most children are diagnosed with Wilms tumour, which has a 90% long-term survival rate with conventional front-line and salvage therapies. However, treatments and outcomes of children with relapsed non-Wilms tumours, such as malignant rhabdoid tumour of the kidney, renal-cell carcinoma (including renal medullary carcinoma), clear-cell sarcoma of the kidney, anaplastic sarcoma of the kidney and congenital mesoblastic nephroma are not well defined. Several of these non-Wilms tumours include unfavourable prognostic subtypes. Currently available data on non-Wilms tumours support advancing studies that pivot from conventional strategies towards disease-specific, biologically driven novel treatments to improve outcomes for each of these rare childhood kidney tumours.</p>

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Relapsed rhabdoid tumours and other non-nephroblastoma childhood and adolescent kidney tumours: perspectives from the HARMONICA collaboration

  • 期刊论文

  • 发表日期：

    2026年1月2日

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  • Michael V. Ortiz,
  • Francis S.P.L. Wens,
  • Andrew L. Hong,
  • Paola Quarello,
  • Arnauld C. Verschuur,
  • Elisabeth T. Tracy,
  • Najat C. Daw,
  • Jeffrey S. Dome,
  • Amy L. Walz,
  • Julia Sprokkerieft,
  • Godelieve A. M. Tytgat,
  • Alex Kentsis,
  • Jaime Libes-Bander,
  • Peter J. Schoettler,
  • Daniela Perotti,
  • Jarno Drost,
  • Rajkumar Venkatramani,
  • James I. Geller,
  • Marry M. van den Heuvel-Eibrink

摘要

Paediatric kidney tumours are generally associated with a favourable survival rate. Most children are diagnosed with Wilms tumour, which has a 90% long-term survival rate with conventional front-line and salvage therapies. However, treatments and outcomes of children with relapsed non-Wilms tumours, such as malignant rhabdoid tumour of the kidney, renal-cell carcinoma (including renal medullary carcinoma), clear-cell sarcoma of the kidney, anaplastic sarcoma of the kidney and congenital mesoblastic nephroma are not well defined. Several of these non-Wilms tumours include unfavourable prognostic subtypes. Currently available data on non-Wilms tumours support advancing studies that pivot from conventional strategies towards disease-specific, biologically driven novel treatments to improve outcomes for each of these rare childhood kidney tumours.