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. 1989 Sep;60(3):358–365. doi: 10.1038/bjc.1989.285

Non-ocular cancer in relatives of retinoblastoma patients.

B M Sanders 1, M Jay 1, G J Draper 1, E M Roberts 1
PMCID: PMC2247167  PMID: 2789942

Abstract

A series of 1,438 parents and 2,663 other relatives of retinoblastoma patients have been followed up to ascertain the incidence among them of non-ocular cancer. Among 117 of these relatives who were known carriers of the mutation of the retinoblastoma gene 23 cases of non-ocular cancer developed during the follow-up period of the study. This compares with an expected number of 2.3, a relative risk of 9.9. A total of 25 deaths among these carriers included 21 from non-ocular cancer; the expected number was 1.8, a relative risk of 11.6. Relatives who are carriers are about 15 times more likely to die from lung cancer than the general population. Previous findings of an association of melanoma and bladder cancer with retinoblastoma are borne out in this study. The incidence of non-ocular cancer among relatives of hereditary cases who are not definitely known to be carriers shows an excess risk of 1.6: it is concluded that a proportion of these relatives are in fact carriers of the mutated retinoblastoma gene. For relatives who are not gene carriers there appears to be no excess risk of developing cancer. Carriers relatives who are not themselves affected with retinoblastoma may be inherently less liable than affected carriers to the further genetic changes which lead to the development of both retinoblastoma and subsequent non-ocular cancer.

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Selected References

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

  1. Abramson D. H., Ellsworth R. M., Kitchin F. D., Tung G. Second nonocular tumors in retinoblastoma survivors. Are they radiation-induced? Ophthalmology. 1984 Nov;91(11):1351–1355. doi: 10.1016/s0161-6420(84)34127-6. [DOI] [PubMed] [Google Scholar]
  2. 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]
  3. Coleman M., Douglas A., Hermon C., Peto J. Cohort study analysis with a FORTRAN computer program. Int J Epidemiol. 1986 Mar;15(1):134–137. doi: 10.1093/ije/15.1.134. [DOI] [PubMed] [Google Scholar]
  4. DerKinderen D. J., Koten J. W., Nagelkerke N. J., Tan K. E., Beemer F. A., Den Otter W. Non-ocular cancer in patients with hereditary retinoblastoma and their relatives. Int J Cancer. 1988 Apr 15;41(4):499–504. doi: 10.1002/ijc.2910410405. [DOI] [PubMed] [Google Scholar]
  5. Draper G. J., Sanders B. M., Kingston J. E. Second primary neoplasms in patients with retinoblastoma. Br J Cancer. 1986 May;53(5):661–671. doi: 10.1038/bjc.1986.110. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Fedrick J., Baldwin J. A. Incidence of cancer in relatives of children with retinoblastoma. Br Med J. 1978 Jan 14;1(6105):83–84. doi: 10.1136/bmj.1.6105.83. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Friend S. H., Bernards R., Rogelj S., Weinberg R. A., Rapaport J. M., Albert D. M., Dryja T. P. A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma. Nature. 1986 Oct 16;323(6089):643–646. doi: 10.1038/323643a0. [DOI] [PubMed] [Google Scholar]
  8. Gordon H. Family studies in retinoblastoma. Birth Defects Orig Artic Ser. 1974;10(10):185–190. [PubMed] [Google Scholar]
  9. Harbour J. W., Lai S. L., Whang-Peng J., Gazdar A. F., Minna J. D., Kaye F. J. Abnormalities in structure and expression of the human retinoblastoma gene in SCLC. Science. 1988 Jul 15;241(4863):353–357. doi: 10.1126/science.2838909. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Knudson A. G., Jr Retinoblastoma: a prototypic hereditary neoplasm. Semin Oncol. 1978 Mar;5(1):57–60. [PubMed] [Google Scholar]
  11. Lee E. Y., To H., Shew J. Y., Bookstein R., Scully P., Lee W. H. Inactivation of the retinoblastoma susceptibility gene in human breast cancers. Science. 1988 Jul 8;241(4862):218–221. doi: 10.1126/science.3388033. [DOI] [PubMed] [Google Scholar]
  12. Matsunaga E. Hereditary retinoblastoma: delayed mutation or host resistance? Am J Hum Genet. 1978 Jul;30(4):406–424. [PMC free article] [PubMed] [Google Scholar]
  13. Murphree A. L., Benedict W. F. Retinoblastoma: clues to human oncogenesis. Science. 1984 Mar 9;223(4640):1028–1033. doi: 10.1126/science.6320372. [DOI] [PubMed] [Google Scholar]
  14. Strong L. C., Herson J., Haas C., Elder K., Chakraborty R., Weiss K. M., Majumder P. Cancer mortality in relatives of retinoblastoma patients. J Natl Cancer Inst. 1984 Aug;73(2):303–311. doi: 10.1093/jnci/73.2.303. [DOI] [PubMed] [Google Scholar]
  15. Tarkkanen A., Karjalainen K. Excess of cancer deaths in close relatives of patients with bilateral retinoblastoma. Ophthalmologica. 1984;189(3):143–146. doi: 10.1159/000309401. [DOI] [PubMed] [Google Scholar]
  16. Yokota J., Akiyama T., Fung Y. K., Benedict W. F., Namba Y., Hanaoka M., Wada M., Terasaki T., Shimosato Y., Sugimura T. Altered expression of the retinoblastoma (RB) gene in small-cell carcinoma of the lung. Oncogene. 1988 Oct;3(4):471–475. [PubMed] [Google Scholar]

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