Retinoblastoma Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (08/09/2017)

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.

General Information About Retinoblastoma

Revised text to state that death from a second neoplasm is the most common cause of death and contributes to about 50% of deaths in patients with bilateral disease and genetically defined heritable retinoblastoma (cited Temming et al. as reference 22).

Added text to state that in a German series of 643 patients with heritable retinoblastoma, chemotherapy with or without radiation therapy was the only significant risk factor for the onset of second cancers outside the periorbital region.

Staging and Grouping Systems for Retinoblastoma

Added text to state that a retrospective German study found that the International Retinoblastoma Staging System (IRSS) predicted survival in 633 children with heritable retinoblastoma, of which 582 patients presented with IRSS stage 0 or I disease (cited Temming et al. as reference 3).

Treatment Option Overview for Retinoblastoma

Added Friedman et al. as reference 16 and level of evidence 2Di and Brennan et al. as reference 17 and level of evidence 3iiA.

Added text about the results of a single-institution, nonrandomized study of 345 patients diagnosed with Group D or E tumors (cited Berry et al. as reference 22 and level of evidence 3iiDii).

Added text to state that some centers have reported higher ocular salvage rates for unilateral Group D and E eyes using a three-drug chemotherapy regimen.

Revised text to state that pilot studies suggested that direct intravitreal injection of melphalan or topotecan may be effective in controlling active vitreous seeds (cited Shields et al. [Retina 2016] as reference 52 and level of evidence 3iiDiv).

Added text to state that the current era of retinoblastoma management using intra-arterial chemotherapy plus intravitreal chemotherapy has improved globe salvage in eyes with advanced retinoblastoma (cited Shields et al. [J Pediatr Ophthalmol Strabismus 2016] as reference 56 and level of evidence 3iiDii).

Treatment of Intraocular Retinoblastoma

Revised text to state that the delivery of chemotherapy via ophthalmic artery cannulation as initial treatment for advanced unilateral retinoblastoma appears to be more effective than does systemic chemotherapy for chemoreduction, particularly for Group D eyes (cited Abramson et al. as reference 13 and Shields et al. as reference 14 and level of evidence 3iiDiv). Also revised text to state that in the setting of a multidisciplinary state-of-the-art center, intra-arterial chemotherapy may result in ocular salvage rates of approximately 80% to 90% for patients with advanced intraocular unilateral retinoblastoma.

Added text to state that similar results were achieved in one single-institution study when topotecan was substituted for etoposide in a combination regimen (cited Brennan et al. as reference 30 and level of evidence 3iiA).

Treatment of Progressive or Recurrent Retinoblastoma

Added Treatment Options Under Clinical Evaluation for Progressive or Recurrent Retinoblastoma as a new subsection.

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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