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Hereditary Wilms Tumor

MedGen UID:
Concept ID:
Neoplastic Process
Synonyms: Familial Wilms Tumor; Familial Wilms' Tumor; Familiar Wilms Tumor; Hereditary Kidney Adenosarcoma; Hereditary Kidney Adenosarcoma (WT1); Hereditary Nephroblastoma; Hereditary Nephroblastoma (WT1); Hereditary Renal Adenosarcoma; Hereditary Renal Adenosarcoma (WT1); Hereditary Wilms' Tumor; Hereditary Wilms' Tumor (WT1)


Familial embryonal neoplasm derived from nephrogenic blastemal cells. Several lines of differentiation, including blastemal, stromal and epithelial, are usually expressed. Comprises approximately 1% of Wilms tumors. (AFIP fascicle version 2.0 mo011000) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHereditary Wilms Tumor

Recent clinical studies


Li FP, Williams WR, Gimbrere K, Flamant F, Green DM, Meadows AT
Pediatrics 1988 Jan;81(1):147-9. PMID: 2827098

Clinical prediction guides

Szabó J, Heath B, Hill VM, Jackson CE, Zarbo RJ, Mallette LE, Chew SL, Besser GM, Thakker RV, Huff V
Am J Hum Genet 1995 Apr;56(4):944-50. PMID: 7717405Free PMC Article
Henry I, Grandjouan S, Couillin P, Barichard F, Huerre-Jeanpierre C, Glaser T, Philip T, Lenoir G, Chaussain JL, Junien C
Proc Natl Acad Sci U S A 1989 May;86(9):3247-51. PMID: 2566168Free PMC Article

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