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E. Chong, M.Y. Hurwitz, R.L. Hurwitz, P. Chevez-Barrios; Necrotic Retinoblastoma: A Clinicopathologic Review . Invest. Ophthalmol. Vis. Sci. 2003;44(13):1573.
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Purpose: To correlate the histopathologic characteristics of enucleated eyes with the clinical presentation and outcome for retinoblastoma patients with extensive tumor necrosis. Methods: Sixty-seven eyes enucleated from patients diagnosed with retinoblastoma at Baylor College of Medicine between 1990 and 2001 were reviewed. Extensive tumor necrosis was detected in eyes from 11 patients. Clinical details were obtained from corresponding medical charts and referring physicians. Results: Only 5 of 11 patients presented with typical leukocoria. The other six presented with red, inflamed eyes, often with chemosis and neovascular glaucoma. Nine of 11 patients had optic nerve invasion; 7 of 11 had choroidal involvement; 9 of 11 had cataracts and 6 of 11 had iris neovascularization. Histopathogic features associated with extensive tumor necrosis included vascular thrombosis, necrotic intraocular structures, iris neovascularization and cataracts. Despite negative surgical margins in all the patients, 2 of the 11 patients died of metastatic disease. Conclusion: Retinoblastoma confined to the eye but demonstrating extensive necrosis often has an atypical presentation. Associations with choroidal and optic nerve involvement portend a worse prognosis for survival despite negative surgical margins. Clinicians should be especially aggressive in the metastatic workup and treatment of these patients
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