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Show Tal/ mal of Clinical Nel/ ro- ophthalmology 9( 3): 222- 223, 1989. Feature Photo Trilateral Retinoblastoma Jerry A. Shields, M. D., Samuel R. Pesin, M. D., and Carol L. Shields, M. D. © 1989 Raven Press, Ltd., New York A l- week- old girl was referred to us because her father and half- sister had been treated for bilateral retinoblastoma. Fundus examination under anesthesia disclosed a solitary retinoblastoma in the right macula and two similar tumors in the left macula, all measuring < 3 mm in diameter. Computed tomography of the brain revealed no evidence of intracranial tumor. The tumors in both eyes regressed after 4,000 cGy ( rad) of external beam radiotherapy. Follow- up examinations under anesthesia every 3 months disclosed normal optic discs and complete control of the retinoblastomas. At the age of 3 years, the child first complained of headaches. Examination under anesthesia revealed continued excellent control of the retinoblastomas, but bilateral papilledema was present ( Figs. 1 and 2). Computed tomography revealed normal eyes, but there was marked hydrocephalus ( Fig. 3) and a large pineal tumor ( Fig. 4). Despite irradiation, chemotherapy, and a craniotomy with a shunting procedure, she died 7 months later with widespread central nervous system involvement by the pineal tumor. The association of bilateral retinoblastoma with midline intracranial neoplasms has become well recognized in recent years. This association is termed " trilateral retinoblastoma" ( 1,2). Genetic counseling for any patient with the familial form of retinoblastoma should include discussion of the From the Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia. Address correspondence and reprint requests to Dr. J. A. Shields, Director. Ocular Oncology Servicl', Wills Eye Hospital, ~ Iinfh , n,: Phd." j, II_' h;". I'A 1<) 107, U. s. A. 222 FIG. 1. Fundus photograph of right eye showing optic disc edema and small residual retinoblastoma in foveal area ( arrows). FIG. 2. Fundus photograph of left eye showing optic disc edema. TRILATERAL RETINOBLASTOMA 223 FIG. 3. Cranial computed tomogram showing dilated ventricles. possibility that midline intracranial neoplasms may develop in that patient ( 1- 5) or family members ( 4,5). Most midline intracranial neoplasms have proved fatal, but it is hoped that earlier detection of the tumor by computed tomography or magnetic resonance imaging will bring about earlier therapeutic intervention and control in the future ( 5). Acknowledgment: This work was supported in part by the Ocular Oncology Fund and the Oncology Research Fund, Philadelphia, and in part by the Black Patch Invitational Golf Tournament, Downingtown, Pennsylvania. FIG. 4. Cranial computed tomogram through pineal region showing pinealoblastoma ( P). REFERENCES 1. Bader Jl, Meadows AT, Zimmerman lE, et al. Bilateral retinoblastoma and ectopic intracranial retinoblastoma: trilateral retinoblastoma. Cancer Genet Cytogenet 1982; 5: 20313. 2. Bader Jl, Miller RW, Meadows AT, Zimmerman LE, Champion lAA, Voute PA. Trilateral retinoblastoma. Lancet 1980; 2: 582- 3. 3. Kingston JE, Plowman PN, Hungerford JL. Ectopic intracranial retinoblastoma in childhood. Br' Ophthalmol 1985; 69: 742~. 4. Zimmerman LE. Trilateral retinoblastoma. In: Blodi FC, ed. Retinoblastoma. New York: Churchill Livingstone, 1985: 185210. 5. Pesin SR, Shields JA. Seven cases of trilateral retinoblastoma. Am I Ophthalmol 1989; 107: 121-{ i. I Clin Neuro- ophthalmol, Vol. 9, No. 3, 1989 |
