Retinoblastoma in Turkey--treatment and prognosis

The treatment modalities and prognosis of 636 retinoblastoma (RB) cases diagnosed and treated in our specialist center between 1963 and 1994 were evaluated. Patient age ranged from 20 days to 16 years, the mean age being 2.2 years (26.4 months). Of the 636 cases, 441 were unilateral and 195 were bilateral. Enucleation was the most frequent treatment employed in unilateral RB patients (412 cases). Follow-up treatment included exenteration (48 cases), radiotherapy (154 cases) and chemotherapy (108 cases) for cases with optic nerve invasion and/or orbital recurrence following enucleation. Seventeen cases displayed massive proptosis, ocular damage and blindness at initial presentation and underwent exenteration as the initial treatment. Two cases were subjected to external beam radiotherapy without invasive surgical procedures. Ten cases regressed spontaneously without treatment. For bilateral cases, the most frequent treatment used was enucleation for one eye and radiotherapy for the other (132 cases). Adjuvant treatment included exenteration (9 cases) and chemotherapy (50 cases) depending on orbital recurrence and/or systemic metastasis. Spontaneous bilateral regression was noted in one case. Six cases underwent bilateral external beam radiotherapy without surgery. One eye of the remaining 56 bilateral cases underwent enucleation. The treatment for the contralateral eyes included cryotherapy in 14 cases, enucleation in 11 cases, Cobalt plaque (Co plaque) therapy in 10 cases, photocoagulation in 6 cases and exenteration in one case. No treatment was undertaken in the contralateral eyes of 14 cases. Secondary treatment modalities employed in these 56 bilateral cases were radiotherapy (11 cases), chemotherapy (8 cases), Co plaque (8 cases) and exenteration (5 cases). Treatment complications were detected in 25 cases followed for at least 18 months. Eighteen cases had radiation cataracts and 6 of these 18 patients underwent intraocular lens implantation. Post-radiation orbital malignancy (osteosarcoma) was noted in two cases aged 14 and 15 years. Phthisis bulbi was observed in three cases and radiation keratitis in two cases. The overall survival rate was 82.2% after a mean follow-up of 5 years. The survival rate of unilateral cases was 82.8% and that of bilateral cases was 81.1% at 5 years.