Abstract
In a series of 882 retinoblastoma patients, 384 known to have the genetic form of the disease and 498 others, 30 patients developed second primary neoplasms. The spectrum of these second neoplasms is discussed in relation to the forms of treatment used for the retinoblastoma. Cumulative incidence rates of second tumours in the whole series are 2.0% at 12 years after diagnosis and 4.2% after 18 years. For patients with the genetic form of retinoblastoma the cumulative incidence rate after 18 years is 8.4% for all second neoplasms and 6.0% for osteosarcomas alone. The inherent risk among survivors from genetic retinoblastoma of developing an osteosarcoma, excluding all possible effects of treatment, is estimated to be 2.2% after 18 years. Within the field of radiation treatment the cumulative incidence rate for all second neoplasms after 18 years is 6.6% and for osteosarcomas alone 3.7%. There is some evidence that patients with genetic retinoblastoma are particularly sensitive to the carcinogenic effects of radiation. The results also suggest that the use of cyclophosphamide may increase the risk of second primary neoplasms in patients with genetic retinoblastoma. The incidence rates of second primary neoplasms in retinoblastoma survivors reported here are lower than those quoted for previously published series. Evidence from this and other papers strongly suggests an association between retinoblastoma and malignant melanoma.
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Selected References
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- 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]
- Abramson D. H., Ellsworth R. M., Zimmerman L. E. Nonocular cancer in retinoblastoma survivors. Trans Sect Ophthalmol Am Acad Ophthalmol Otolaryngol. 1976 May-Jun;81(3 Pt 1):454–457. [PubMed] [Google Scholar]
- Abramson D. H., Ronner H. J., Ellsworth R. M. Second tumors in nonirradiated bilateral retinoblastoma. Am J Ophthalmol. 1979 May;87(5):624–627. doi: 10.1016/0002-9394(79)90293-9. [DOI] [PubMed] [Google Scholar]
- Aherne G. Retinoblastoma associated with other primary malignant tumours. Trans Ophthalmol Soc U K. 1974;94(4):938–944. [PubMed] [Google Scholar]
- Bader J. L., Meadows A. T., Zimmerman L. E., Rorke L. B., Voute P. A., Champion L. A., Miller R. W. Bilateral retinoblastoma with ectopic intracranial retinoblastoma: trilateral retinoblastoma. Cancer Genet Cytogenet. 1982 Mar;5(3):203–213. doi: 10.1016/0165-4608(82)90026-7. [DOI] [PubMed] [Google Scholar]
- 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]
- 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]
- François J. Conservative treatment of retinoblastoma. Mod Probl Ophthalmol. 1977;18:113–117. [PubMed] [Google Scholar]
- François J. Retinoblastoma and osteogenic sarcoma. Ophthalmologica. 1977;175(4):185–191. doi: 10.1159/000308656. [DOI] [PubMed] [Google Scholar]
- François J., de Sutter E., Coppieters R., de Bie S. Late extraocular tumours in retinoblastoma survivors. Ophthalmologica. 1980;181(2):93–99. doi: 10.1159/000309033. [DOI] [PubMed] [Google Scholar]
- Hoefnagel D., McIntyre O. R., Storrs R. C., Sullivan P. B., Maurer L. H. Retinoblastoma followed by acute lymphoblastic leukemia. Lancet. 1973 Mar 31;1(7805):725–725. doi: 10.1016/s0140-6736(73)91517-1. [DOI] [PubMed] [Google Scholar]
- Jensen R. D., Miller R. W. Retinoblastoma: epidemiologic characteristics. N Engl J Med. 1971 Aug 5;285(6):307–311. doi: 10.1056/NEJM197108052850602. [DOI] [PubMed] [Google Scholar]
- Kingston J. E., Plowman P. N., Hungerford J. L. Ectopic intracranial retinoblastoma in childhood. Br J Ophthalmol. 1985 Oct;69(10):742–748. doi: 10.1136/bjo.69.10.742. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Kitchin F. D., Ellsworth R. M. Pleiotropic effects of the gene for retinoblastoma. J Med Genet. 1974 Sep;11(3):244–246. doi: 10.1136/jmg.11.3.244. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Knudson A. G., Jr Retinoblastoma: a prototypic hereditary neoplasm. Semin Oncol. 1978 Mar;5(1):57–60. [PubMed] [Google Scholar]
- LELE K. P., PENROSE L. S., STALLARD H. B. CHROMOSOME DELETION IN A CASE OF RETINOBLASTOMA. Ann Hum Genet. 1963 Nov;27:171–174. doi: 10.1111/j.1469-1809.1963.tb00209.x. [DOI] [PubMed] [Google Scholar]
- Magnasco A., Zingirian M., Cottafava F. Su un raro caso di leucemia istioide a rapida evoluzione, insorta dopo trattamento roeentgenterapico ed antiblastico per retinoblastoma. Ann Ottalmol Clin Ocul. 1967 Mar;93(3):300–308. [PubMed] [Google Scholar]
- Meadows A. T., Baum E., Fossati-Bellani F., Green D., Jenkin R. D., Marsden B., Nesbit M., Newton W., Oberlin O., Sallan S. G. Second malignant neoplasms in children: an update from the Late Effects Study Group. J Clin Oncol. 1985 Apr;3(4):532–538. doi: 10.1200/JCO.1985.3.4.532. [DOI] [PubMed] [Google Scholar]
- Meadows A. T., Strong L. C., Li F. P., D'Angio G. J., Schweisguth O., Freeman A. I., Jenkin R. D., Morris-Jones P., Nesbit M. E. Bone sarcoma as a second malignant neoplasm in children: influence of radiation and genetic predisposition for the Late Effects Study Group. Cancer. 1980 Dec 15;46(12):2603–2606. doi: 10.1002/1097-0142(19801215)46:12<2603::aid-cncr2820461212>3.0.co;2-4. [DOI] [PubMed] [Google Scholar]
- 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]
- Nuutinen J., Kärjä J., Sainio P. Epithelial second malignant tumours in retinoblastoma survivors. A review and report of a case. Acta Ophthalmol (Copenh) 1982 Feb;60(1):133–140. doi: 10.1111/j.1755-3768.1982.tb05789.x. [DOI] [PubMed] [Google Scholar]
- Peto R., Pike M. C., Armitage P., Breslow N. E., Cox D. R., Howard S. V., Mantel N., McPherson K., Peto J., Smith P. G. Design and analysis of randomized clinical trials requiring prolonged observation of each patient. I. Introduction and design. Br J Cancer. 1976 Dec;34(6):585–612. doi: 10.1038/bjc.1976.220. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Sagerman R. H., Cassady J. R., Tretter P., Ellsworth R. M. Radiation induced neoplasia following external beam therapy for children with retinoblastoma. Am J Roentgenol Radium Ther Nucl Med. 1969 Mar;105(3):529–535. doi: 10.2214/ajr.105.3.529. [DOI] [PubMed] [Google Scholar]
- Schmähl D., Habs M., Lorenz M., Wagner I. Occurrence of second tumors in man after anticancer drug treatment. Cancer Treat Rev. 1982 Sep;9(3):167–194. doi: 10.1016/s0305-7372(82)80006-6. [DOI] [PubMed] [Google Scholar]
- 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]
- Strong L. C., Herson J., Osborne B. M., Sutow W. W. Risk of radiation-related subsequent malignant tumors in survivors of Ewing's sarcoma. J Natl Cancer Inst. 1979 Jun;62(6):1401–1406. [PubMed] [Google Scholar]
- Tefft M., Vawter G. F., Mitus A. Second primary neoplasms in children. Am J Roentgenol Radium Ther Nucl Med. 1968 Aug;103(4):800–822. doi: 10.2214/ajr.103.4.800. [DOI] [PubMed] [Google Scholar]
- Vogel F. Genetics of retinoblastoma. Hum Genet. 1979 Nov 1;52(1):1–54. doi: 10.1007/BF00284597. [DOI] [PubMed] [Google Scholar]
- Weichselbaum R. R., Nove J., Little J. B. Skin fibroblasts from a D-deletion type retinoblastoma patient are abnormally X-ray sensitive. Nature. 1977 Apr 21;266(5604):726–727. doi: 10.1038/266726a0. [DOI] [PubMed] [Google Scholar]
- White L., Ortega J. A., Ying K. L. Acute non-lymphocytic leukemia following multimodality therapy for retinoblastoma. Cancer. 1985 Feb 1;55(3):496–498. doi: 10.1002/1097-0142(19850201)55:3<496::aid-cncr2820550303>3.0.co;2-u. [DOI] [PubMed] [Google Scholar]