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J. A. Chen, J. T. Wilkinson, S. McCaffery, K. K. Matthay, J. M. O'Brien; Optimal Duration of Chemotherapy in Retinoblastoma: The UCSF Experience. Invest. Ophthalmol. Vis. Sci. 2007;48(13):5255.
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From a database of over 400 retinoblastoma patients managed at UCSF, a subset of patients was selected to determine if systemic chemoreduction and local ophthalmic therapy is successful in avoiding external beam radiation therapy (EBRT) and unplanned enucleation in the treatment of retinoblastoma.
Retrospective case review.
Patients who received chemotherapy for retinoblastoma from 1992-2005 at University of California San Francisco were retrospectively identified. Patients included in the study were managed surgically by a single ophthalmologist. Patients who received any form of treatment elsewhere were excluded in order to study patients receiving uniform therapy. Only patients receiving primary chemotherapy with etoposide, vincristine and carboplatin were included. Median follow-up time was 61 months (range 7 months to 136 months).
A total of 36 patients/56 eyes met stringent study criteria. Eyes were staged as Group A (2), Group B (8), Group C (12), Group D (12), and Group E (22) based on the International Classification System for Retinoblastoma. A total of 23 eyes received planned enucleation (all 22 Group E eyes and 1 Group D eye). Of these 23 eyes, 15 received adjuvant chemotherapy after a planned enucleation, 4 eyes received chemoreduction to shrink their tumors prior to planned enucleation, and 4 eyes received chemoreduction both prior and following planned enucleation. The remaining 33 eyes (Groups A through D) received chemotherapy as neo-adjuvant therapy in addition to local ophthalmic therapy. Of these 33 eyes, 9 eyes (27%) received unplanned enucleation and/or EBRT after chemotherapy. Five eyes (15%) received EBRT, 2 eyes (6%) received unplanned enucleation, and 2 eyes (6%) received a combination of EBRT and enucleation. The median number of cycles of chemoreduction when used as neo-adjuvant therapy was 8.5 (range 3 to 22). All 36 patients treated with chemotherapy for retinoblastoma survived.
Systemic chemoreduction in combination with local ophthalmic therapy is an effective technique to treat retinoblastoma in children. Aggressive use of chemotherapy, often more than the 6 cycles of chemoreduction currently recommended by the Children’s Oncology Group protocols for neo-adjuvant therapy, may be beneficial in avoiding external beam radiation therapy and unplanned enucleation.
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