Retinoblastoma in Turkey: results from a tertiary care center in Ankara

To evaluate the presentation patterns and results of management of retinoblastoma at a tertiary care center in Ankara, Turkey, with special emphasis on globe conservation rate in unilateral and bilateral intraocular retinoblastoma.

Patients were grouped according to the International Classification of Retinoblastoma. For intraocular retinoblastoma, group E and some group D eyes underwent primary enucleation. Secondary enucleation was performed after failure of chemoreduction, focal treatments, external beam radiotherapy (EBRT), and intra-arterial chemotherapy used in various combinations. For extraocular retinoblastoma cases, treatment consisted of enucleation/exenteration or orbital biopsy, high-dose chemotherapy, and EBRT to the orbit and metastatic sites.

During the study period from October 1998 to May 2010, 165 of 192 (85.9%) patients had intraocular disease and 27 (14.1%) patients had extraocular disease. In total, primary or secondary enucleation was performed in 70 of 94 eyes with unilateral retinoblastoma (74.5%) and in 34 of 142 eyes with bilateral retinoblastoma (23.9%). The overall globe conservation rate was 69.6%. Only one patient in the intraocular retinoblastoma group died of metastatic retinoblastoma to the central nervous system. Twenty of 27 patients (74.1%) with extraocular retinoblastoma were found to have metastasis to the central nervous system, bone, bone marrow, and/or lymph nodes. At a mean follow-up of 28.0 months (median: 12 months; range: 1 to 120 months), survival was 33.3% despite intensive treatment.

The overall risk of enucleation was 75% in eyes with unilateral retinoblastoma and 24% in eyes with bilateral retinoblastoma. Extraocular retinoblastoma carries a 75% risk of systemic metastasis and 67% risk of death.