Retinoblastoma: experience of a referral center in the North Region of Portugal

PURPOSE To describe the experience of the Ophthalmology Department of Hospital São João (HSJ), a tertiary health care center in North Region, Portugal, in terms of the diagnosis, treatment, and follow-up of retinoblastoma. METHODS This was a retrospective study of patients diagnosed with retinoblastoma in Hospital São João, between 1978 and 2012. RESULTS Fifty patients with retinoblastoma were evaluated in our institution between 1978 and 2012. Four patients were excluded due to loss of follow-up. Among the 46 retinoblastoma cases, 33 (71.7%) were unilateral and 13 (28.3%) bilateral, with a mean age at diagnosis of 22.19 months and 6.92 months, respectively (P<0.001). Leukocoria was the most common presenting sign (36.9%), followed by strabismus (19.6%), a combination of leukocoria and strabismus (8.7%), and buphthalmia (2.2%). Between 1978 and 1992, nine retinoblastoma cases were referred to our hospital, all of them unilateral, and, in each case, enucleation was performed, with or without salvage therapy. Between 1993 and 2012, 18 eyes with retinoblastoma were successfully managed with conservative treatment. CONCLUSION Demographic results were generally coincident with previous reports. It is crucial to screen leukocoria in pediatric practice, even in asymptomatic children. The outcome of retinoblastoma treatment in our hospital is similar to other series in developed countries.