Kidney Wilms tumor

MedGen UID:: 989515
•Concept ID:: CN305775
•: Disease or Syndrome

Synonym:	Wilms Tumor of the Kidney

Related genes: Gene(s) associated with related conditions. For conditions in a hierarchy, the parent condition will list the genes associated with the children conditions.	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]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVNephroblastoma
- CROGVKidney Wilms tumor
  - CROGVHereditary Wilms tumor

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Neoplasm
      - Embryonal neoplasm
        Nephroblastoma
        Kidney Wilms tumor
        Hereditary Wilms tumor
        Wilms tumor 1
        Wilms tumor 2
        Wilms tumor 3
        Wilms tumor 4
        Wilms tumor 5

Professional guidelines

PubMed

Wilms Tumor (Nephroblastoma), Version 2.2021, NCCN Clinical Practice Guidelines in Oncology.

Balis F, Green DM, Anderson C, Cook S, Dhillon J, Gow K, Hiniker S, Jasty-Rao R, Lin C, Lovvorn H, MacEwan I, Martinez-Agosto J, Mullen E, Murphy ES, Ranalli M, Rhee D, Rokitka D, Tracy EL, Vern-Gross T, Walsh MF, Walz A, Wickiser J, Zapala M, Berardi RA, Hughes M
J Natl Compr Canc Netw 2021 Aug 1;19(8):945-977. doi: 10.6004/jnccn.2021.0037. PMID: 34416707

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: 30310143 Free PMC Article

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