Paediatric metanephric tumours: a clinicopathological and molecular characterisation

Dominique V C de Jel, Janna A Hol, Ariadne H A G Ooms, Ronald R de Krijger, Marjolijn C J Jongmans, Annemieke S Littooij, Jarno Drost, Martine van Grotel, Marry M van den Heuvel-Eibrink

Research output: Contribution to journalArticlepeer-review


To characterize metanephric tumours in children, we performed a literature review investigating paediatric metanephric adenomas (MA), metanephric stromal tumours (MST) and metanephric adenofibromas (MAF). Including two patients from our own institution (MA, MAF), 110 individual cases (41 MA, 20 MAF, 49 MST) were identified. Additionally, fifteen composite tumours were identified, with areas of MA/MAF and Wilms tumour (WT) or papillary carcinoma. No distinct clinical or radiological features could be defined. In pure metanephric tumours, histologically proven distant metastases were reported once (MA), relapse was reported once (MST) and one tumour-related death occurred (MST). Somatic BRAF-V600E mutations were tested in 15 cases, and identified in 3/6 MA, 3/3 MAF, and 6/6 MST. In our institution the MA harboured a somatic KRAS-G12R mutation. Overall, paediatric metanephric tumours are difficult to discriminate from other renal tumours at presentation, behave relatively benign, and the occurrence of composite tumours warrants analysis of underlying (genetic) pathways.

Original languageEnglish
Pages (from-to)102970
JournalCritical reviews in oncology/hematology
Publication statusPublished - Jun 2020


  • Adenoma/genetics
  • Biomarkers, Tumor/genetics
  • Carcinoma, Papillary/genetics
  • Child
  • DNA Mutational Analysis
  • Humans
  • Immunohistochemistry
  • Immunophenotyping
  • Kidney Neoplasms/genetics
  • Neoplasm Recurrence, Local
  • Proto-Oncogene Proteins p21(ras)/genetics
  • Wilms Tumor/genetics


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