Purchase this article with an account.
G.M. Freeman, L.F. Teixeira, C.M. Erwenne, C. Macedo; Intraocular Retinoblastoma: Reese–Ellsworth Group Vb–an Epidemiologic Study and Results of Treatment . Invest. Ophthalmol. Vis. Sci. 2006;47(13):2292.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
To describe the results of treatment in all new cases of bilateral retinoblastoma in whom at least one eye was Reese–Ellsworth Group Vb on initial examination, seen at the above location between 1 January 2001 and 30 June 2005.
This was a retrospective chart review and data were collected on age, sex, family history, eyes affected, delay to treatment and followup time. Treatment modalities used included where indicated: initial chemoreduction (in all but one case whose eye was enucleated primarily), transpupillary thermotherapy, cryotherapy, plaque radiotherapy, external beam radiotherapy (EBR) and enucleation. Outcome measures were number of eyes saved, number of enucleations, and deaths.
There were a total of 96 new untreated patients (131 eye affected) during this period. Of these, 20 patients (7 male and 13 female, with 9 and 19 eyes totalling 28) were classified as Group Vb. Three patients died (15%) representing 3 eyes lost: one a result of alternative therapy used after parents abandoned our care and two with sepsis treated elsewhere. There were 15 eyes enucleated (15/25=60%). There were 10 eyes salvaged (40%). Of these 5 were saved without EBR and 5 with it. Mean age at EBR was 30.2 months. ( EBR was not used for any unilateral Vb case.) Mean followup was 19.4 months .Mean delay to treatment was 4.4 months.
Assuming that bilateral (germ line) retinoblastoma is different in behavior from unilateral (somatic) disease, we examined only bilateral cases of Group Vb eyes. We saved 40 % of all eyes, but only 20% without the use of external beam radiotherapy after chemoreduction and focal treatment. Thus, EBR near the end of treatment significantly improved the salvage rate in our group. However, our salvage rate is still less than that reported by Kingston and Hungerford (1996) of almost 70 % with both chemoreduction and EBR, and less than those of Abramson (2004) of 48%, with one course of EBR as the only therapy.We found no second tumours during followup; our death rate of 15% and delay to treatment of 4.4 months are still higher than those figures from first world countries.This study, to our knowledge, represents the first report in the English literature dealing with only bilateral R–E Group Vb patients treated initially with chemoreduction and focal therapy, and EBR afterwards if needed.
This PDF is available to Subscribers Only