Purchase this article with an account.
C. L. Weiss, N. Bornfeld; Regression Patterns and Visual Acuity of Macular Tumours Following Chemoreduction for Retinoblastoma. Invest. Ophthalmol. Vis. Sci. 2010;51(13):2072.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
In the last 15 years the therapy for retinoblastoma changed totally since it is known that there is a high risk for secondary malagnnacies for children with a germline mutation in the RB 1 gene after external beam radiation (EBRT). Systemic chemotherapy with focal treatment (chemoreduction) is now firstline treatment in the majority of cases without vitreous seeding. However, macular tumours are still a big challenge, as treatment may severly compromise visual function and quality of life.
In a retrospective case study 54 tumours of 50 eyes and 40 childeren were evaluated treated with chemotherappy (vincristine, carbopplatin, etoposid every 3 weeks for on average 6 times) plus focal treatment (preferably TCT) between 1996 and 2008. Only tumours within the vascular arcades were included. The distance to the fovea and the optic disc was measured for every single tumour on base of the digital pictures taken with the Ret Cam.
The average follow up was 88months (median 84 months). The distance to the fovea was on average 0.8mm (median 0.7mm) and to the optic disc 1.6mm. 28 tumours (52%) had a relapse or were still active after or at the end of chemoreduction. 25 (89%) of these tumours were inactive at the end of follow up. In 9 tumours of 8 patients external beam radiation was needed.The majority of the patients showed a type 3 regression pattern (23 tumours, 46%) or I (15 tumours, 27%) after chemotherapy. 9 tumours (16%) still had signs of activity. The regression patterns changed during treatment because of additional focal treatment: at the end of follow up 39% (22 tumours) had a regression pattern IV. The visual acuity was on average 0.8log(Mar) (media 0.9 logMar).
Systemic chemotherapy and additional focal treatment (chemoreduction) have a lower local tumour control than EBRT of tumours within the vessel arcades. Insiting on chemotherapy is the only solution now available to avoid a high rate of secondary malagnancies. A new therapy strategy must be developed in order to guarantee a tumour control similar to EBRT and saving the vision important structure parts. There are new options like chemosurgery giving an oppurtunity for further investigations that coul lead to better regression- and vision results of these tumours.
This PDF is available to Subscribers Only