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Hereditary Wilms tumor

MedGen UID:
146190
Concept ID:
C0677779
Neoplastic Process
Synonym: Hereditary Wilms' tumor
 
Related genes: H19-ICR, H19, POU6F2, WT1, IGF2, GPC3, GPC4, BRCA2
 
Monarch Initiative: MONDO:0003321
OMIM® Phenotypic series: PS194070

Definition

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) [from NCI]

Professional guidelines

PubMed

Rao A, Rothman J, Nichols KE
Curr Opin Pediatr 2008 Feb;20(1):1-7. doi: 10.1097/MOP.0b013e3282f4249a. PMID: 18197032
Pakakasama S, Tomlinson GE
Pediatr Clin North Am 2002 Dec;49(6):1393-413. doi: 10.1016/s0031-3955(02)00095-0. PMID: 12580371
Garber JE, Diller L
Curr Opin Pediatr 1993 Dec;5(6):712-5. doi: 10.1097/00008480-199312000-00014. PMID: 8124424

Recent clinical studies

Etiology

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. doi: 10.1073/pnas.86.9.3247. PMID: 2566168Free PMC Article

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