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Expert Rev Mol Med. 2017 Jul 18;19:e8. doi: 10.1017/erm.2017.8.

Bilateral Wilms tumour: a review of clinical and molecular features.

Author information

1
UCL Institute of Child Health,University College London,London,UK.
2
Centre Léon Bérard,Institut d'Hématologie et d'Oncologie Pédiatrie,Lyon,France.

Abstract

Wilms tumour (WT) is the most common paediatric kidney cancer and affects approximately one in 10 000 children. The tumour is associated with undifferentiated embryonic lesions called nephrogenic rests (NRs) or, when diffuse, nephroblastomatosis. WT or NRs can occur in both kidneys, termed bilateral disease, found in only 5-8% of cases. Management of bilateral WT presents a major clinical challenge in terms of maximising survival, preserving renal function and understanding underlying genetic risk. In this review, we compile clinical data from 545 published cases of bilateral WT and discuss recent progress in understanding the molecular basis of bilateral WT and its associated precursor NRs in the context of the latest radiological, surgical and epidemiological features.

PMID:
28716159
PMCID:
PMC5687181
DOI:
10.1017/erm.2017.8
[Indexed for MEDLINE]
Free PMC Article

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