• Walentyna Balwierz, Danuta Pietrys, Bozena Romanowska-Dixon, Joanna Kobylarz, Katarzyna Pawińska-Wasikowska, and Angelina Moryl-Bujakowska.
• Klinika Onkologii i Hematologii Dzieciecej, PA Instytut Pediatrii UJ-CM, Kraków. balwierz@mp.pl
• Prz. Lek. 2010 Jan 1;67(6):404-8.

AbstractUntil recently chemotherapy was used as adjuvant therapy after enucleation in cases with extraretinal spread of the disease (uveal extension, orbital extension, neoplastic infiltrates of the optic nerve at resection line, intracranial metastasis, generalized disease). Recent experience has proved that use of chemotherapy for intraocular retinoblastoma before local treatment (so called "chemoreduction") has allowed not only to decrease number of enucleations and indications for external beam irradiation or limit the extension of local therapy, but also increase chances for vision preservation and decrease the risk of severe complications. Seventy five children (with 106 involved eyes) aged 0.2 - 106 months with intraocular retinoblastoma diagnosed between January 1996 and June 2009 were the subject of this study. Among 106 involved eyes, in 70 (66%) the V stage according to Reese-Ellsworth classification (R-E) was established. Enucleation before chemotherapy was necessary in 9 (8.5%) cases, and in 22 more children the eye had to be removed after 1-2 courses of chemotherapy. In 68 remaining children (with 70 involved eyes) VEC (vincristine, etoposide, carboplatin) chemotherapy combined with delayed local therapy (cryotherapy, photocoagulation, brachytherapy) was employed. Out of 84 eyes treated by combined methods eye enucleation could be avoided in 47 (67%), including 18 (90%), 13 (87%) and 16 (46%) qualified to R-E group I-II, III-IV and V, respectively. First-line chemotherapy combined with the local treatment should be standard treatment for intraocular retinoblastoma groups I - IV. More effective therapy is required for R-E eye group V cases.

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