Paediatric metanephric tumours: a clinicopathological and molecular characterisation.
adenofibroma
adenoma
metanephric
paediatric
renal
stromal tumour
Journal
Critical reviews in oncology/hematology
ISSN: 1879-0461
Titre abrégé: Crit Rev Oncol Hematol
Pays: Netherlands
ID NLM: 8916049
Informations de publication
Date de publication:
Jun 2020
Jun 2020
Historique:
received:
29
07
2019
revised:
05
02
2020
accepted:
15
04
2020
pubmed:
7
5
2020
medline:
17
6
2020
entrez:
7
5
2020
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 32371339
pii: S1040-8428(20)30108-6
doi: 10.1016/j.critrevonc.2020.102970
pii:
doi:
Substances chimiques
Biomarkers, Tumor
0
KRAS protein, human
0
Proto-Oncogene Proteins p21(ras)
EC 3.6.5.2
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
102970Informations de copyright
Copyright © 2020 The Author(s). Published by Elsevier B.V. All rights reserved.