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Retinoblastoma: experience of a referral center in Northwestern Portugal

Session Details

Session Title: Uveitis & Intraocular Tumours

Session Date/Time: Friday 27/09/2013 | 14:30-16:00

Paper Time: 15:34

Venue: Hall C (Level 1)

First Author: P.Freitas-da-Costa PORTUGAL

Co Author(s):    P. Costa   J. Araujo   T. Braganca   E. Brandao     

Abstract Details

Purpose:

In this study we aim to describe the experience of the Ophthalmology Department of Hospital S. João in the diagnosis, treatment and follow-up of retinoblastoma.

Setting:

Study performed at Hospital S. João, Porto, Portugal (HSJ, a terceary health-care center).

Methods:

This is a retrospective study in which we reviewed the clinical files of patients diagnosed with retinoblastoma who were referred to HSJ between 1978 and 2012. All cases were simultaneously evaluated by an Oncology-specialized Ophthalmologist and Pediatric Oncology experts from the Pediatric Department. From 1993, every local conservative treatments were performed in Centre Ophthalmic Jules Gonin, Lausanne-Switzerland. The patients were staged according to Reese and Ellsworth grading system

Results:

Fifty patients with retinoblastoma were evaluated in our institution between 1978 and 2012. Four patients were excluded from the analysis due to loss of follow-up. The male to female ratio was 1:1. The mean follow-up period was 12 years, ranging from 1 to 33 years. Among the total of 46 retinoblastoma cases, 33 (71,7%) were unilateral and 13 (28,3%) were bilateral. The mean age at diagnosis was 22,19 months for unilateral retinoblastomas versus 6,92 months for bilateral cases, and this difference achieved statistical significance (p<0,001). Leukocoria was the most common presenting sign, being found in 54,83% of cases, followed by strabism (29,3%), combination of leukocoria and strabism (12,9%) and buphthalmia (3,22%). 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 out of 50 eyes (36%) with retinoblastoma were successfully treated with conservative therapy alone. We recorded one death during this follow-up period, concerning a case of a stage V unilateral retinoblastoma, treated with enucleation, chemo and radiotherapy (due to choroidal and optic nerve invasion).

Conclusions:

All the demographic results of this study were generally coincident with previous reports concerning retinoblastoma laterality, gender distribution and age of diagnosis described worldwide. Leukocoria was the most frequent presenting sign described by the parents and referring doctors. Attending to the easy access to this test and its major importance in the early diagnosis of retinoblastoma, it becomes crucial for every doctor to search for leukocoria in their pediatric practice, even in asymptomatic children. The mortality rate of retinoblastoma in our hospital is similar to the expected in developed countries. Concerning retinoblastoma treatment, we’ve come a long way in the last decades overcoming three different and consecutive goals: saving the patient’s life; saving the patient’s eye; saving the patient’s vision.

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