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Carol L. Shields, MD; Arman Mashayekhi, MD; Hakan Demirci, MD; Anna T. Meadows, MD; Jerry A. Shields, MD

Arch Ophthalmol. 2004;122:729-735.

Objective  To present a simplified approach to management of retinoblastoma using basic clinical features.

Design  In a prospective, nonrandomized, single-center clinical trial, 158 eyes of 103 patients with retinoblastoma were managed with 6 cycles of chemoreduction (vincristine sulfate, etoposide, and carboplatin). The eyes were classified according to the Reese-Ellsworth classification and were also grouped on the basis of clinical features as follows: group 1, tumor only; group 2, tumor plus subretinal fluid; group 3, tumor plus focal seeds (3a, focal subretinal seeds; 3b, focal vitreous seeds); group 4, tumor plus diffuse seeds (4a, diffuse subretinal seeds; 4b, diffuse vitreous seeds); and group 5, neovascular glaucoma or invasive retinoblastoma.

Main Outcome Measure  Treatment success (avoidance of enucleation and external beam radiotherapy).

Results  According to the Reese-Ellsworth classification, chemoreduction was successful in 100% of group Ia, 100% of group Ib, 86% of group IIa, 100% of group IIb, 91% of group IIIa, 100% of group IIIb, 50% of group IVa, 77% of group IVb, 50% of group Va, and 27% of group Vb. There was erratic correlation of the Reese-Ellsworth classification with treatment success. In contrast, the simplified grouping system displayed a smooth, nonerratic correlation for treatment success, with 100% success for group 1, 91% for group 2, 59% for group 3, and 12% for group 4 (group 5 always managed by primary enucleation). When all 6 subcategory groups were analyzed, there was consistent correlation for treatment success of 100% for group 1, 91% for group 2, 68% for group 3a, 54% for group 3b, 17% for group 4a, and 11% for group 4b.

Conclusion  This practical approach to retinoblastoma using basic clinical features is predictive of treatment success for eyes in which modern conservative therapy for retinoblastoma is used.

From the Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University (Drs C. L. Shields, Mashayekhi, Demirci, and J. A. Shields), and Division of Oncology, The Children's Hospital of Philadelphia (Dr Meadows), Philadelphia, Pa. The authors have no relevant financial interest in this article.

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