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Eye Cancer Network Case #13: Retinoblastoma
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Leukocoria with tumor visible through the right pupil. Note the clear cornea and lack of orbital signs.
History: This 16-month old patient was referred for bilateral leukocoria (white pupil), acute glaucoma OS, with evidence of anterior chamber seeding and possible optic nerve/orbital invasion. The left pupil was nonreactive.
 The left eye is enlarged. The cornea is opacified, the conjunctiva is injected, while tumor cells can be seen on the corneal endothelium (arrows).
Computed tomography demonstrates intratumoral calcifications consistent with retinoblastoma (black arrows). The left optic nerve is enlarged and retrobulbar opacities suggest extrascleral extension (white arrow).
Impression: Retinoblastoma
*Note* This risks and benefits of observation, enucleation, chemotherapy and external beam radiation therapy were discussed in detail. Single agent cisplatinum chemotherapy (3 cycles due to definite choroidal and possible orbital and optic nerve involvement) and enucleation of the left eye was performed. Histopathologic evaluation of the left eye revealed retinoblastoma without optic nerve or orbital extension. The right eye was enucleated after failure of systemic chemoreduction to produce a treatable tumor.
Comment: This case presents multiple classic findings of retinoblastoma: leukocoria (white pupil), secondary glaucoma, orbital inflammation, anterior segment seeding, strabismus, and intraocular calcification. Other findings of retinoblastoma (not found in this case) can include pinealoma, and extraocular tumor extension. In North America today, when the tumor is confined to the eye more than 95% of children survive their retinoblastoma.
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