Retinoblastoma Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (01/25/2018)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Treatment Option Overview for Retinoblastoma

Added text to state that in a retrospective study of Group D eyes that were treated primarily with intravenous chemotherapy and never enucleated, more than 50% of patients had visual acuity of less than 20/200 and 75% of patients had strabismus (cited Fabian et al. as reference 23).

Added text to state that the addition of intravitreal chemotherapy to intra-arterial chemotherapy appears to markedly improve overall treatment effectiveness in eyes with vitreous seeds, especially those with vitreous seed clouds (cited Francis, Iyer, et al. as reference 43).

Added text about how a proportion of patients with abnormal electroretinograms (ERGs) with or without retinal detachment may have improved ERGs in the years after intra-arterial chemotherapy, including the results of one study that included 72 eyes with advanced retinoblastoma (cited Abdelhakim et al. as reference 49).

Revised text to state that a meta-analysis reported that significant side effects of intravitreal chemotherapy are uncommon, although each injection results in a small decrease in retinal function, as measured by ERG; the current era of retinoblastoma management using intra-arterial chemotherapy plus intravitreal chemotherapy has improved globe salvage in eyes with advanced retinoblastoma when compared with children who were treated in earlier years with intra-arterial chemotherapy alone. Also added text about the results of a retrospective study that addressed 40 eyes with vitreous seed clouds that were treated in two different eras (added level of evidence 3iiDiv). Also added text to state that generally, each dose of intravitreal chemotherapy results in a further small decrement of retinal function, as measured by ERG (cited Francis, Brodie, et al. as reference 59).

Treatment of Intraocular Retinoblastoma

Added text to state that high-risk pathology has been associated with the presence of minimal dissemination in bone marrow and cerebrospinal fluid using quantitative polymerase chain reaction for detection of cone-rod homeobox or GD2 synthase. In a group of 96 children with nonmetastatic retinoblastoma and high-risk pathology, the 3-year disease-free survival was 78% for patients with detectable minimal dissemination, compared with 98% for those without detectable disease (cited Laurent et al. as reference 7).

Added Munier et al. as reference 15.

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

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