GENETICS AND EPIDEMIOLOGY OF WILMS-TUMOR - THE FRENCH WILMS-TUMOR STUDY

被引:35
作者
BONAITIPELLIE, C
CHOMPRET, A
TOURNADE, MF
HOCHEZ, J
MOUTOU, C
ZUCKER, JM
STESCHENKO, D
BRUNATMENTIGNY, M
ROCHE, H
TRON, P
FRAPPAZ, D
MUNZER, M
BACHELOT, C
DUSOL, F
SOMMELETOLIVE, D
LEMERLE, J
机构
[1] INST CURIE, F-75231 PARIS 05, FRANCE
[2] INST GUSTAVE ROUSSY, F-94805 VILLEJUIF, FRANCE
[3] HOP ENFANTS, NANCY, FRANCE
[4] CTR LEON BERARD, F-69373 LYONS, FRANCE
[5] CTR CLAUDIUS REGAUD, F-31052 TOULOUSE, FRANCE
[6] HOP CHARLES NICOLLE, F-76031 ROUEN, FRANCE
[7] HOP NORD ST ETIENNE, ST ETIENNE, FRANCE
[8] HOP AMER, REIMS, FRANCE
[9] CTR HOSP REG & UNIV, GRENOBLE, FRANCE
[10] CTR OSCAR LAMBRET, F-59020 LILLE, FRANCE
来源
MEDICAL AND PEDIATRIC ONCOLOGY | 1992年 / 20卷 / 04期
关键词
FAMILY HISTORY; GENETICS; WILMS TUMOR; FRANCE;
D O I
10.1002/mpo.2950200404
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
A complete family history was obtained for 501 patients with Wilms' tumor, treated in departments of pediatric oncology in whole France. The information was collected by self-questionnaire and/or by interview of parents. The proportion of bilateral cases is 4.6% and there are 12 patients (2.4%) with a positive family history of Wilms' tumor. The affected relatives are most often distant and no first degree relative was affected. Apart from the well-known associations with aniridia, hemihypertrophy, genitourinary anomalies, Beckwith-Wiedeemann, and Drash syndromes, there is also a significant excess of congenital heart defects (P = .008) which remains to be explained. Several findings support the bimutational hypothesis such as earlier diagnosis and increased parental age in bilateral cases. No particular anomalies and no increased frequency of childhood cancer were found in patients' relatives. The frequency of Wilms' tumor in relatives was estimated to be less than 0.4% in sibs, 0.06% in uncles and aunts, and 0.04% in first cousins. These figures are very different from those found in retinoblastoma and suggest that the mechanism may be more complex in Wilms' tumor. This conclusion is in agreement with molecular biology studies in tumors and linkage analysis in multiple case families which suggest that more than one locus is involved.
引用
收藏
页码:284 / 291
页数:8
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