Full Report

Format

Full Report
Summary (Text)
Summary (XML)

Send to:

Choose Destination

File
Clipboard
Collections

Hereditary Wilms tumor

MedGen UID:: 146190
•Concept ID:: C0677779
•: Neoplastic Process

Synonym:	Hereditary Wilms' tumor

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: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]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVNephroblastoma
- CROGVKidney Wilms tumor
  - CROGVHereditary Wilms tumor

Pathological process
- Disease
  - Neoplasm
    - Malignant neoplastic disease
      - Hereditary cancer
        Hereditary Malignant Urinary System Neoplasm
        Hereditary Wilms tumor
        Wilms tumor 1
        Wilms tumor 2
        Wilms tumor 3
        Wilms tumor 4
        Wilms tumor 5

Professional guidelines

PubMed

Genetic testing and tumor surveillance for children with cancer predisposition syndromes.

Rao A, Rothman J, Nichols KE
Curr Opin Pediatr 2008 Feb;20(1):1-7. doi: 10.1097/MOP.0b013e3282f4249a. PMID: 18197032

Genetic predisposition and screening in pediatric cancer.

Pakakasama S, Tomlinson GE
Pediatr Clin North Am 2002 Dec;49(6):1393-413. doi: 10.1016/s0031-3955(02)00095-0. PMID: 12580371

Screening children at genetic risk of cancer.

Garber JE, Diller L
Curr Opin Pediatr 1993 Dec;5(6):712-5. doi: 10.1097/00008480-199312000-00014. PMID: 8124424

See all (5)

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Wilms Tumor (Nephroblastoma), 2023

Recent clinical studies

Etiology

Heritable fraction of unilateral Wilms tumor.

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

See all (1)

Clinical prediction guides

Hereditary hyperparathyroidism-jaw tumor syndrome: the endocrine tumor gene HRPT2 maps to chromosome 1q21-q31.

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: 7717405 Free PMC Article

Tumor-specific loss of 11p15.5 alleles in del11p13 Wilms tumor and in familial adrenocortical carcinoma.

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: 2566168 Free PMC Article

See all (2)

MedGen

National Center for Biotechnology Information

Send to:

Hereditary Wilms tumor

Definition

Term Hierarchy

Professional guidelines

PubMed

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Wilms Tumor (Nephroblastoma), 2023

Recent clinical studies

Etiology

Clinical prediction guides

Supplemental Content

Table of contents

Genetic Testing Registry

Clinical resources

Practice guidelines

Curated

Consumer resources

Reviews

Related information

Recent activity