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M. W. Wilson, C. Billups, B. G. Haik, C. Rodriguez-Galindo; A Clinicopathological Correlation of 67 Eyes Primarily Enucleated for Advanced Intraocular Retinoblastoma. Invest. Ophthalmol. Vis. Sci. 2009;50(13):1683.
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To correlate clinical and histopathological findings of eyes primarily enucleated for advanced intraocular retinoblastoma.
A retrospective study identified patients primarily enucleated for retinoblastoma. Patient demographics and clinical findings (sex, age at diagnosis, laterality of disease, corneal diameter, intraocular pressure, Reese- Ellsworth Classification, and International Classification) at diagnosis were retrieved from the medical record. Subsequent treatments and outcomes were also recorded. The histopathology of each eye was reviewed for invasion of the iris, ciliary body, choroid, sclera and optic nerve and extraocular extension.
We identified 67 eyes of 67 patients (33 males) primarily enucleated for retinoblastoma between March 1997 and January 2008. The majority of patients (n=62) had unilateral disease. Median age at diagnosis was 24 months (range, 1.5 - 190). All eyes were classified as Reese-Ellsworth Group V (Va = 19 and Vb = 48) and International Classification Group D (n = 47) or Group E (n=19). Median corneal diameter was 12 mm (range, 9-14) and median intraocular pressure was 22 mmHg (range, 9-64). The majority of eyes had some degree of choroidal (focal n=24, massive n=17) and optic nerve (pre-laminar n=28, laminar n=21, post-laminar n=9) invasion. Five eyes had iris invasion, 7 had ciliary body invasion and 10 had scleral invasion. Extraocular extension was noted in one eye. Corneal diameter, intraocular pressure, and Reese-Ellsworth Classification had no significant association with invasive disease. The International Classification, however, was associated with optic nerve (p = 0.026), choroid (p < 0.001), ciliary body (p = 0.002), iris (p = 0.002) and scleral (p < 0.001) invasion. Eyes classified as International Classification Group E were more likely to have invasion of these sites and have more severe optic nerve invasion. 17 patients required additional treatment (chemotherapy n=17 and radiation n=1). 2 patients died from metastatic disease. Median follow was 39 months (range,1-129).
Corneal diameter, intraocular pressure, and Reese-Ellsworth Classification do not correlate with histological evidence of invasive retinoblastoma. Eyes classified as International Classification Group E are more likely to have invasion of the uveal tract, optic nerve and sclera. Our findings warrant meticulous histopathological examination of such eyes.
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