This article is more than 5 years old. Information may no longer be current.

Retinoblastoma survivors at long-term risk for death from subsequent malignant neoplasms

Add topic to email alerts

Receive an email when new articles are posted on

Please provide your email address to receive an email when new articles are posted on .

Subscribe

Added to email alerts

You've successfully added to your alerts. You will receive an email when new content is published.

Click Here to Manage Email Alerts

You've successfully added to your alerts. You will receive an email when new content is published.

Click Here to Manage Email Alerts

Back to Healio

We were unable to process your request. Please try again later. If you continue to have this issue please contact customerservice@slackinc.com.

Back to Healio

Patients who survive hereditary retinoblastoma may be at increased risk for death from subsequent sarcomas, melanomas and cancers of the brain and nervous system as many as 40 years after their retinoblastoma diagnosis, according to follow-up data from a retrospective cohort study. Additionally, patients treated with radiotherapy were at a greater risk compared with those who were not.

The study, a follow-up from a 1990 mortality report, included 1,854 hereditary and nonhereditary retinoblastoma survivors diagnosed between Jan. 1, 1914, and Dec. 31, 1996. Using U.S. mortality data, the researchers estimated expected numbers of death and calculated standardized mortality ratios. They determined relative rates for mortality due to subsequent neoplasms and calculated cumulative mortality from subsequent neoplasms.

At the end of follow-up in December 2003, 65.9% of hereditary retinoblastoma and 86.6% of nonhereditary retinoblastoma survivors were alive. Compared with the general population, retinoblastoma survivors did not have excess mortality from non-neoplastic causes. However, among hereditary retinoblastoma survivors excess mortality from subsequent malignant neoplasms extended more than 40 years after diagnosis.

Thirteen years of follow-up showed an increased risk for death from cancers of the corpus uteri and confirmed previously reported risks for death from lung cancer among hereditary retinoblastoma survivors.

The cumulative mortality rate from subsequent neoplasms at 50 years was higher among hereditary retinoblastoma survivors (25.5%; 95% CI, 20.8%-30.2%) compared with nonhereditary retinoblastoma survivors (1.0%; 95% CI, 0.2%-1.8%).

“The temporal patterns of site-specific excess risks of subsequent malignant neoplasms in retinoblastoma survivors should inform screening programs designed for the early detection and treatment of subsequent malignant neoplasms,” the researchers wrote.

Yu CL. J Natl Cancer Inst. 2009;101:581-591.