PMID- 8827065
OWN - NLM
STAT- MEDLINE
DCOM- 19961105
LR  - 20161019
IS  - 0098-1532 (Print)
IS  - 0098-1532 (Linking)
VI  - 27
IP  - 5
DP  - 1996 Nov
TI  - Familial Wilms' tumor: a descriptive study.
PG  - 398-403
AB  - Among 6,209 patients with Wilms' tumor entered on the National Wilms' Tumor Study 
      (NWTS), 93 patients (1.5%) from 63 families had a positive family history. In 30 
      of these 63 families a (half) sibling or parent of the NWTS patient was confirmed 
      to have had Wilms' tumor. Fifteen (16.1%) of the familial, but only 7.1% of 
      sporadic cases, had bilateral disease. Mean ages at diagnosis were 15.8 vs. 35.2 
      months (P = 0.012) for bilateral vs. unilateral familial cases and 32.0 vs. 44.7 
      months for sporadic cases. Intralobar nephrogenic rests were found twice as 
      frequently in association with the tumors of familial as with those of sporadic 
      cases. Cases of bilateral and metastatic disease tended to cluster within 
      specific families, suggesting heterogeneity in the genetic etiology. The number 
      and age distribution of familial cases transmitted through the father were about 
      the same as those of cases transmitted through the mother. This finding is 
      inconsistent with models of genomic imprinting that involve familial transmission 
      of a tumor-suppressor gene and it casts further doubt on the hypothesis that all 
      bilateral cases are hereditary.
FAU - Breslow, N E
AU  - Breslow NE
AD  - Department of Biostatistics, University of Washington, Seattle 98195-7232, USA.
FAU - Olson, J
AU  - Olson J
FAU - Moksness, J
AU  - Moksness J
FAU - Beckwith, J B
AU  - Beckwith JB
FAU - Grundy, P
AU  - Grundy P
LA  - eng
GR  - R01 CA054498/CA/NCI NIH HHS/United States
GR  - P30 CA 16056/CA/NCI NIH HHS/United States
GR  - R01 CA54498/CA/NCI NIH HHS/United States
GR  - UIO CA 42326/CA/NCI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Med Pediatr Oncol
JT  - Medical and pediatric oncology
JID - 7506654
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Age Distribution
MH  - Age Factors
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - Follow-Up Studies
MH  - Genes, Wilms Tumor/genetics
MH  - Genomic Imprinting
MH  - Humans
MH  - Infant
MH  - Kidney Neoplasms/*genetics/pathology
MH  - Male
MH  - Neoplasm Staging
MH  - Registries
MH  - Survival Rate
MH  - Treatment Outcome
MH  - Wilms Tumor/*genetics/pathology
MH  - X Chromosome/genetics
MH  - Y Chromosome/genetics
EDAT- 1996/11/01 00:00
MHDA- 2000/06/20 09:00
CRDT- 1996/11/01 00:00
PHST- 1996/11/01 00:00 [pubmed]
PHST- 2000/06/20 09:00 [medline]
PHST- 1996/11/01 00:00 [entrez]
AID - 10.1002/(SICI)1096-911X(199611)27:5<398::AID-MPO2>3.0.CO;2-H [pii]
AID - 10.1002/(SICI)1096-911X(199611)27:5<398::AID-MPO2>3.0.CO;2-H [doi]
PST - ppublish
SO  - Med Pediatr Oncol. 1996 Nov;27(5):398-403. doi: 
      10.1002/(SICI)1096-911X(199611)27:5<398::AID-MPO2>3.0.CO;2-H.