Changes to This Summary (10/05/2012)

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

Revised text to state that the RB1 germline mutation may have been inherited from an affected progenitor or may have occurred in utero at the time of conception, in patients with sporadic disease. Also revised text to state that the penetrance of the mutation is probably dependent on concurrent genetic modifiers such as MDM2 and MDM4 (cited de Oliveira et al. as reference 5).

Added text about how early-in-life screening by fundus exams under general anesthesia at regular intervals can improve prognosis in terms of globe sparing in children with positive family histories of retinoblastoma (cited Rothschild et al. as reference 10).

The Subsequent neoplasms subsection was renamed from Second malignant neoplasms.

Revised text to state that more recent studies analyzing cohorts of patients treated with more advanced radiation planning and delivery technology have reported the incidence to be about 9.4% in nonirradiated patients and about 30.4% in irradiated patients.

Stage Information

Revised text to state that extraocular retinoblastoma may be confined to the tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system, bone marrow, or lymph nodes (metastatic retinoblastoma).

Intraocular Retinoblastoma Treatment

This section was extensively revised.

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.