Abstract
A 5-day-old female patient was found to have large hereditary retinoblastomas in the posterior pole of each eye. The patient received radiation treatment over a 39-day period, with each retina receiving 4600 rad. Two weeks after the complete treatment the tumours had regressed to approximately one-quarter of their original size. By 14 weeks following completion of radiotherapy the patient had developed in each eye extensive iris neovascularisation with progressive closure of the filtration angles, secondary glaucoma, and retinal detachments resulting from fibrovascular proliferation on the retinal surface. Radiosensitivity studies were from separate conjunctival biopsies obtained before and after radiation. These showed a D0 (calculated survival curve parameters, defined in the Methods section) in the exponential growth phase of 110 prior to radiation and a postirradiation exponential growth phase D0 of 70. Karotype studies showed several chromosomal abnormalities following radiotherapy. The clinical course and pathology findings are thought to represent an unusually severe orbital and ocular response to radiation therapy. These findings are consistent with our hypothesis that some patients with hereditary retinoblastoma may have a defect in the accumulation repair of x-irradiation induced DNA damage.
Full text
PDF
Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Egbert P. R., Donaldson S. S., Moazed K., Rosenthal A. R. Visual results and ocular complications following radiotherapy for retinoblastoma. Arch Ophthalmol. 1978 Oct;96(10):1826–1830. doi: 10.1001/archopht.1978.03910060338008. [DOI] [PubMed] [Google Scholar]
- Howard G. M. Ocular effects of radiation and photocoagulation. A study of 100 globes with retinoblastoma, some of which were treated prior to enucleation. Arch Ophthalmol. 1966 Jul;76(1):7–10. doi: 10.1001/archopht.1966.03850010009004. [DOI] [PubMed] [Google Scholar]
- Miller D. A., Firschein I. L., Dev V. G., Tantravahi R., Miller O. J. The gorilla karyotype: chromosome lengths and polymorphisms. Cytogenet Cell Genet. 1974;13(6):536–550. doi: 10.1159/000130305. [DOI] [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]
- Shukovsky L. J., Fletcher G. H. Retinal and optic nerve complications in a high dose irradiation technique of ethmoid sinus and nasal cavity. Radiology. 1972 Sep;104(3):629–634. doi: 10.1148/104.3.629. [DOI] [PubMed] [Google Scholar]
- Spaulding G. Rubeosis iridis in retinoblastoma and pseudoglioma. Trans Am Ophthalmol Soc. 1978;76:584–609. [PMC free article] [PubMed] [Google Scholar]
- WINTER F. C., REINHARDT P. R., MADDEN J. Ocular effects of high intensity gamma radiation in the cat. Am J Ophthalmol. 1958 Jul;46(1 Pt 2):114–122. doi: 10.1016/0002-9394(58)90043-6. [DOI] [PubMed] [Google Scholar]
- Walton D. S., Grant W. M. Retinoblastoma and iris neovascularization. Am J Ophthalmol. 1968 Apr;65(4):598–599. doi: 10.1016/0002-9394(68)93882-8. [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]
- Weichselbaum R. R., Nove J., Little J. B. X-ray sensitivity of diploid fibroblasts from patients with hereditary or sporadic retinoblastoma. Proc Natl Acad Sci U S A. 1978 Aug;75(8):3962–3964. doi: 10.1073/pnas.75.8.3962. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Weichselbaum R. R., Nove J., Little J. B. X-ray sensitivity of fifty-three human diploid fibroblast cell strains from patients with characterized genetic disorders. Cancer Res. 1980 Mar;40(3):920–925. [PubMed] [Google Scholar]