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Journal of Medical Genetics logoLink to Journal of Medical Genetics
. 1994 Jun;31(6):429–434. doi: 10.1136/jmg.31.6.429

The French Wilms' tumour study: no clear evidence for cancer prone families.

C Moutou 1, J Hochez 1, A Chompret 1, M F Tournade 1, C Le Bihan 1, J M Zucker 1, J Lemerle 1, C Bonaïti-Pellié 1
PMCID: PMC1049918  PMID: 8071968

Abstract

Wilms' tumour of the kidney is known to occur in Beckwith-Wiedemann syndrome. It has also been described in four cancer prone families displaying Li-Fraumeni syndrome but it is not usually considered to be part of this syndrome. In order to detect particular familial cancer aggregations associated with this tumour, we studied the cancer incidence and mortality among relatives of the 501 Wilms' tumour patients in the French Wilms' Tumour Study. We found no familial association with breast cancer or soft tissue sarcomas which are the most common cancers in the Li-Fraumeni syndrome. However, we found two significant familial associations of Wilms' tumour with bone cancers on the one hand and with brain tumours on the other hand. These associations could reflect a small proportion of families segregating for some susceptibility gene. This should then be confirmed at the molecular level.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bonaïti-Pellié C., Briard-Guillemot M. L. Excess of cancer deaths in grandparents of patients with retinoblastoma. J Med Genet. 1980 Apr;17(2):95–101. doi: 10.1136/jmg.17.2.95. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Bonaïti-Pellié C., Chompret A., Tournade M. F., Hochez J., Moutou C., Zucker J. M., Steschenko D., Brunat-Mentigny M., Roché H., Tron P. Genetics and epidemiology of Wilms' tumor: the French Wilms' tumor study. Med Pediatr Oncol. 1992;20(4):284–291. doi: 10.1002/mpo.2950200404. [DOI] [PubMed] [Google Scholar]
  3. Bonaïti-Pellié C., Chompret A., Tournade M. F., Zucker J. M., Lemerle J. Etude génétique et épidémiologique française sur le néphroblastome: résultats préliminaires. Bull Cancer. 1988;75(1):131–133. [PubMed] [Google Scholar]
  4. Breslow N., Olshan A., Beckwith J. B., Green D. M. Epidemiology of Wilms tumor. Med Pediatr Oncol. 1993;21(3):172–181. doi: 10.1002/mpo.2950210305. [DOI] [PubMed] [Google Scholar]
  5. Brown K. W., Williams J. C., Maitland N. J., Mott M. G. Genomic imprinting and the Beckwith-Wiedemann syndrome. Am J Hum Genet. 1990 May;46(5):1000–1001. [PMC free article] [PubMed] [Google Scholar]
  6. Grundy P., Koufos A., Morgan K., Li F. P., Meadows A. T., Cavenee W. K. Familial predisposition to Wilms' tumour does not map to the short arm of chromosome 11. Nature. 1988 Nov 24;336(6197):374–376. doi: 10.1038/336374a0. [DOI] [PubMed] [Google Scholar]
  7. Huff V., Compton D. A., Chao L. Y., Strong L. C., Geiser C. F., Saunders G. F. Lack of linkage of familial Wilms' tumour to chromosomal band 11p13. Nature. 1988 Nov 24;336(6197):377–378. doi: 10.1038/336377a0. [DOI] [PubMed] [Google Scholar]
  8. Knudson A. G., Jr Mutation and cancer: statistical study of retinoblastoma. Proc Natl Acad Sci U S A. 1971 Apr;68(4):820–823. doi: 10.1073/pnas.68.4.820. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Li F. P., Fraumeni J. F., Jr, Mulvihill J. J., Blattner W. A., Dreyfus M. G., Tucker M. A., Miller R. W. A cancer family syndrome in twenty-four kindreds. Cancer Res. 1988 Sep 15;48(18):5358–5362. [PubMed] [Google Scholar]
  10. Li F. P., Fraumeni J. F., Jr Prospective study of a family cancer syndrome. JAMA. 1982 May 21;247(19):2692–2694. [PubMed] [Google Scholar]
  11. Monson R. R. Analysis of relative survival and proportional mortality. Comput Biomed Res. 1974 Aug;7(4):325–332. doi: 10.1016/0010-4809(74)90010-x. [DOI] [PubMed] [Google Scholar]
  12. Moutou C., Chompret A., Hochez J., Tournade M. F., Zucker J. M., Lemerle J., Junien C., Bonaïti-Pellié C. Testing genomic imprinting in Wilm's tumor. Eur J Hum Genet. 1993;1(3):190–205. doi: 10.1159/000472413. [DOI] [PubMed] [Google Scholar]
  13. Moutou C., Junien C., Henry I., Bonaïti-Pellié C. Beckwith-Wiedemann syndrome: a demonstration of the mechanisms responsible for the excess of transmitting females. J Med Genet. 1992 Apr;29(4):217–220. doi: 10.1136/jmg.29.4.217. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Pal N., Wadey R. B., Buckle B., Yeomans E., Pritchard J., Cowell J. K. Preferential loss of maternal alleles in sporadic Wilms' tumour. Oncogene. 1990 Nov;5(11):1665–1668. [PubMed] [Google Scholar]
  15. Riccardi V. M., Sujansky E., Smith A. C., Francke U. Chromosomal imbalance in the Aniridia-Wilms' tumor association: 11p interstitial deletion. Pediatrics. 1978 Apr;61(4):604–610. [PubMed] [Google Scholar]
  16. Schroeder W. T., Chao L. Y., Dao D. D., Strong L. C., Pathak S., Riccardi V., Lewis W. H., Saunders G. F. Nonrandom loss of maternal chromosome 11 alleles in Wilms tumors. Am J Hum Genet. 1987 May;40(5):413–420. [PMC free article] [PubMed] [Google Scholar]
  17. Schwartz C. E., Haber D. A., Stanton V. P., Strong L. C., Skolnick M. H., Housman D. E. Familial predisposition to Wilms tumor does not segregate with the WT1 gene. Genomics. 1991 Aug;10(4):927–930. doi: 10.1016/0888-7543(91)90181-d. [DOI] [PubMed] [Google Scholar]
  18. Van Heyningen V., Hastie N. D. Wilms' tumour: reconciling genetics and biology. Trends Genet. 1992 Jan;8(1):16–21. doi: 10.1016/0168-9525(92)90019-z. [DOI] [PubMed] [Google Scholar]
  19. Williams J. C., Brown K. W., Mott M. G., Maitland N. J. Maternal allele loss in Wilms' tumour. Lancet. 1989 Feb 4;1(8632):283–284. doi: 10.1016/s0140-6736(89)91300-7. [DOI] [PubMed] [Google Scholar]
  20. Zhang Y., Tycko B. Monoallelic expression of the human H19 gene. Nat Genet. 1992 Apr;1(1):40–44. doi: 10.1038/ng0492-40. [DOI] [PubMed] [Google Scholar]

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