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Paediatric renal tumours: an update on challenges and recent developments.

Gino R Somers1,2, Aurore L'Herminé-Coulomb3, Andres Matoso4

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Virchows Archiv : an International Journal of Pathology
|January 9, 2025
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Summary

Paediatric renal tumours encompass diverse neoplasms like nephroblastoma and renal cell carcinoma. Advances in understanding their biology offer therapeutic opportunities, though diagnostic challenges persist for pathologists.

Keywords:
Clear cell sarcoma of kidneyCongenital mesoblastic nephromaMalignant rhabdoid tumourNephroblastomaPaediatric renal tumourRenal cell carcinomaWilms’ tumour

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Area of Science:

  • Pediatric Oncology
  • Nephropathology
  • Cancer Genomics

Background:

  • Paediatric renal tumours present a spectrum of neoplasms distinct from adult kidney cancer.
  • Nephroblastoma is the most common in the first decade, with high cure rates but persistent challenges.
  • Other first-decade tumours include congenital mesoblastic nephroma, clear cell sarcoma, and malignant rhabdoid tumour, some with poor prognoses.

Purpose of the Study:

  • To review diagnostic categorisation of paediatric renal cancers.
  • To highlight how biological insights are informing therapeutic strategies.
  • To discuss ongoing challenges in the pathological diagnosis of these tumours.

Main Methods:

  • Review of current literature on paediatric renal tumour classification and biology.
  • Analysis of diagnostic criteria and molecular characteristics.
  • Discussion of therapeutic implications based on underlying tumour biology.

Main Results:

  • Paediatric renal tumours are classified into embryonal, mesenchymal, and epithelial types, with distinct biological drivers.
  • Molecular characterisation is revealing subtypes of renal cell carcinoma in adolescents.
  • Despite advances, challenges remain in precise diagnosis and treatment stratification.

Conclusions:

  • Understanding the molecular and epigenetic drivers of paediatric renal tumours is crucial for improved diagnostics and therapeutics.
  • Continued research is needed to address the challenges in managing aggressive subtypes and ensuring optimal patient outcomes.
  • Pathological expertise remains central to accurate diagnosis and guiding treatment decisions.