A retrospective review is reported of 64 group D retinoblastoma (RB) eyes (52 patients) treated with primary IVC, between 2002 and 2014 from two centres, in the UK and US. The median age at presentation was 11 months, 35 (67%) patients had bilateral disease, 38 (73%) patients germline mutation disease and eight (15%) were familial. These patients also received a median number of three treatments, including thermotherapy / cryotherapy, plaque radiotherapy, intraophthalmic artery chemotherapy (IAC) and / or intravitreal chemotherapy. External beam radiotherapy (EBRT) was used in five eyes; all were enucleated eventually; 63% of eyes were salvaged over a median follow-up period of 55 months. Globe salvage rate was 83%, 70%, 59% and 45% at one, three, five and 10 years respectively by Kaplan-Meier survival analysis. No metastatic spread or deaths or were found in this series. IVC related adverse events include febrile neutropenia in 21 (40%) and anaphylactic reaction in two (4%) patients; all resolved conservatively. Two (10%) and one (5%) eyes developed third and sixth nerve palsies after IAC. The authors concluded that primary IVC with adjuvant treatment for group D RB is safe and effective, achieving 63% eye salvage rate and no metastatic spread or deaths found in the series. IAC has now replaced EBRT as a successful salvage treatment.
Primary intravenous chemotherapy (IVC) for Group D retinoblastoma
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