The treatment of retinoblastoma with four-drug regimen including cisplatin, etoposide, vincristine, and cyclophosphamide

Abstract

Over an 11-year period, 59 patients (83 eyes) were treated with four-drug chemotherapy (cisplatin, etoposide, cyclophosphamide, and vincristine) at Hacettepe University, Departments of Ophthalmology and Pediatric Oncology. We evaluated the clinical features, treatment modalities, and outcome of these patients with a median follow-up of 55 months (range 9-130 months). Enucleation was performed as a first-line treatment for 30 eyes due to iris neovascularization and neovascular glaucoma, tumor in the anterior chamber regardless of the tumor stage, and for the patients with the Reese-Ellsworth (RE) group Vb. Chemotherapy was given regardless of tumor stages according to the RE groups in all 59 patients (83 eyes). Fifty-three eyes were treated with chemoreduction (CRD) and focal treatment. The rates of globe preservation were 87% for bilateral tumors and 35% for unilateral tumors in the CRD group. The 5-year overall (OS) and enucleation-free survival (EnFS) was 86.9% and 40%, respectively, for the whole group. At 3rd year, ocular survival rate for the eyes with vitreal or subretinal seeding was 58% and without seeding was 66% (P = .78). Seeding or subretinal collection may not indicate poor prognosis under intensive chemotherapy. The intensive four-drug chemotherapy protocol might have satisfactory results in the retinoblastoma (RBL) patients.