TY - JOUR
T1 - Paediatric metanephric tumours
T2 - a clinicopathological and molecular characterisation
AU - de Jel, Dominique V C
AU - Hol, Janna A
AU - Ooms, Ariadne H A G
AU - de Krijger, Ronald R
AU - Jongmans, Marjolijn C J
AU - Littooij, Annemieke S
AU - Drost, Jarno
AU - van Grotel, Martine
AU - van den Heuvel-Eibrink, Marry M
N1 - Copyright © 2020 The Author(s). Published by Elsevier B.V. All rights reserved.
PY - 2020/6
Y1 - 2020/6
N2 - 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.
AB - 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.
KW - adenofibroma
KW - adenoma
KW - metanephric
KW - paediatric
KW - renal
KW - stromal tumour
UR - http://www.scopus.com/inward/record.url?scp=85083884517&partnerID=8YFLogxK
U2 - 10.1016/j.critrevonc.2020.102970
DO - 10.1016/j.critrevonc.2020.102970
M3 - Review article
C2 - 32371339
SN - 1040-8428
VL - 150
JO - Critical Reviews in Oncology/Hematology
JF - Critical Reviews in Oncology/Hematology
M1 - 102970
ER -