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A.C. Schefler, N. Cicciarelli, T.G. Murray; Macular Retinoblastoma: Evaluation of Tumor Control, Local Complications, and Visual Outcomes for Eyes Treated With Aggressive Chemotherapy and Laser Ablation . Invest. Ophthalmol. Vis. Sci. 2005;46(13):1110.
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Purpose: To determine tumor control rates, complication rates, and visual acuity for patients with macular retinoblastoma undergoing aggressive systemic chemotherapy and diode laser ablation. Methods: All patients with retinoblastoma located in the macula treated at the Bascom Palmer Eye Institute between May, 1991 and April, 2004 were retrospectively reviewed. All patients were treated with an planned therapeutic strategy, and managed upon presentation by the same clinician. Patients with Reese–Ellsworth Group 1–4 disease underwent 4–9 cycles of systemic chemotherapy with vincristine, etoposide, and carboplatin and patients with Group 5 disease underwent 6–10 cycles +/– cyclosporin. All tumors underwent repetitive diode laser ablation (2–24 sessions, median 9) at each clinical visit until until tumors were observed to be inactive for approximately 6 months. Results: Forty–five eyes of 34 patients were treated. Eighteen patients (53%) were males and 16 (47%) were females. Six patients (18%) had unilateral retinoblastoma, and twenty–eight patients (82%) had bilateral disease. Eyes were classified as Reese–Ellsworth Group: I: 2%; II: 12%; III: 7%; IV: 9%; V: 70%. Thirty–nine eyes (87%) had successful tumor control and avoided enucleation at a median follow–up of 42 months (range, 7–100). All six eyes requiring enucleation were in Reese–Ellsworth Group 5. No eyes required external beam radiation in order to achieve tumor control. Complications included: iris atrophy (64%), peripheral focal lens opacity (13%), peripheral anterior synechiae (4%), and foveal neovascular membrane (2%). Esotropia was noted at last evaluation in five percent of eyes and exotropia in 32%. A relative afferent pupillary defect was elicited in 47% of eyes evaluated. Snellen visual acuity or Teller acuity card equivalent measured 20/40 or better in 38% of eyes, 20/50 to 20/80 in 19%, and worse than 20/80 in 44%. No patients developed metastatic retinoblastoma or a second malignancy or died within the study interval. Conclusions: Treatment of macular retinoblastoma with chemotherapy and aggressive laser therapy applied repetitively at each clinical visit resulted in tumor control and the avoidance of enucleation in 87% of eyes at a median of 42 months follow–up. All eyes that required enucleation were in Reese–Ellsworth Group 5, and no patients required external beam radiation in order to achieve globe conservation. Patients had a 57% chance of retaining eyes with vision 20/80 or better.
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