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Kidney Wilms tumor

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


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


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
Vujanić GM, Gessler M, Ooms AHAG, Collini P, Coulomb-l'Hermine A, D'Hooghe E, de Krijger RR, Perotti D, Pritchard-Jones K, Vokuhl C, van den Heuvel-Eibrink MM, Graf N; International Society of Paediatric Oncology–Renal Tumour Study Group (SIOP–RTSG)
Nat Rev Urol 2018 Nov;15(11):693-701. doi: 10.1038/s41585-018-0100-3. PMID: 30310143Free PMC Article
Prasad M, Vora T, Agarwala S, Laskar S, Arora B, Bansal D, Kapoor G, Chinnaswamy G, Radhakrishnan V, Kaur T, Rath GK, Bakhshi S
Indian J Pediatr 2017 Jun;84(6):437-445. Epub 2017 Apr 3 doi: 10.1007/s12098-017-2305-5. PMID: 28367612

Recent clinical studies

Clinical prediction guides

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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