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Kidney Wilms tumor

MedGen UID:
989515
•Concept ID:
CN305775
•
Disease or Syndrome
Synonym: Wilms Tumor of the Kidney
 
Related genes: H19-ICR, H19, POU6F2, WT1, IGF2, GPC3, GPC4, BRCA2
 
Monarch Initiative: MONDO:0019004

Definition

An embryonal pediatric tumor of the kidney which may also be seen rarely in adults. The peak incidence of Wilms tumor is between the second and fifth year of life. Microscopically, it is composed of a mixture of cellular elements (blastemal, stromal, and epithelial). The most common sites of metastasis include the regional lymph nodes, lungs, and liver. [from MONDO]

Professional guidelines

PubMed

The Spectrum of PAX6 Mutations and Genotype-Phenotype Correlations in the Eye.
Lima Cunha D, Arno G, Corton M, Moosajee M
Genes (Basel) 2019 Dec 17;10(12) doi: 10.3390/genes10121050. PMID: 31861090Free PMC Article
Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association.
Lipshultz SE, Law YM, Asante-Korang A, Austin ED, Dipchand AI, Everitt MD, Hsu DT, Lin KY, Price JF, Wilkinson JD, Colan SD
Circulation 2019 Jul 2;140(1):e9-e68. Epub 2019 May 28 doi: 10.1161/CIR.0000000000000682. PMID: 31132865
Evidence for a delay in diagnosis of Wilms' tumour in the UK compared with Germany: implications for primary care for children.
Pritchard-Jones K, Graf N, van Tinteren H, Craft A
Arch Dis Child 2016 May;101(5):417-20. Epub 2016 Mar 6 doi: 10.1136/archdischild-2015-309212. PMID: 26948824Free PMC Article

Recent clinical studies

Clinical prediction guides

Identical genetic changes in different histologic components of Wilms' tumors.
Zhuang Z, Merino MJ, Vortmeyer AO, Bryant B, Lash AE, Wang C, Deavers MT, Shelton WF, Kapur S, Chandra RS
J Natl Cancer Inst 1997 Aug 6;89(15):1148-52. doi: 10.1093/jnci/89.15.1148. PMID: 9262253

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