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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

Advances in the clinical management of high-risk Wilms tumors.
Ortiz MV, Koenig C, Armstrong AE, Brok J, de Camargo B, Mavinkurve-Groothuis AMC, Herrera TBV, Venkatramani R, Woods AD, Dome JS, Spreafico F
Pediatr Blood Cancer 2023 May;70 Suppl 2(Suppl 2):e30342. doi: 10.1002/pbc.30342. PMID: 37096797Free 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
The UMBRELLA SIOP-RTSG 2016 Wilms tumour pathology and molecular biology protocol.
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

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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