Retinoblastoma Survivors Face Risk of Second Cancers

Posted on March 8th, 2009 by

Retinoblastoma Survivors Face Risk of Second Cancers

A study that followed retinoblastoma survivors for up to 61 years reported a high risk of second cancers among survivors of hereditary retinoblastoma. These results were published in the Journal of the National Cancer Institute.

Retinoblastoma is a rare cancer of the retina (part of the eye). The disease most commonly occurs in young children. Approximately 30%-40% of cases are due to an inherited mutation in a gene known as RB1. The remaining 60%-70% of cases are thought to be due to sporadic (not inherited) mutations in the RB1 gene.

Hereditary retinoblastoma is suspected if the patient has a family history of retinoblastoma or if the disease involves both eyes. Survivors of hereditary retinoblastoma are known to be at risk of developing second cancers, but few studies have had long-term follow-up information available.

To explore the risk of second cancers among retinoblastoma survivors, including some with several decades of follow-up, researchers in the Netherlands conducted a study among 668 patients who had been diagnosed with retinoblastoma between 1945 and 2005. The risk of cancer in the retinoblastoma survivors was compared to the risk of cancer in the general Dutch population.

  • Among survivors of hereditary retinoblastoma, the risk of cancer was 20-times higher than in the general population. Patients who had received radiation therapy for hereditary retinoblastoma had an even greater increase in subsequent cancer risk.
  • The results suggested that during the first 40 years after a diagnosis of hereditary retinoblastoma, 28% of survivors will develop a second cancer. The types of cancer that were more common among the retinoblastoma survivors than in the general population included bone cancer, soft tissue sarcoma, melanoma, bladder cancer, and lung cancer.
  • Among survivors of nonhereditary retinoblastoma, the risk of cancer was slightly elevated (less than two-times higher than in the general population), but this result did not meet the criteria for statistical significance, suggesting that it could have occurred by chance alone.

This study provides additional information about the risk of second cancers among retinoblastoma survivors. Survivors of hereditary retinoblastoma may wish to talk with their doctor about the optimal approach to follow-up and cancer screening.

Reference: Marees T, Moll AC, Imhof SM, de Boer MR, Ringens PJ, van Leeuwen FE. Risk of second malignancies in survivors of retinoblastoma: more than 40 years of follow-up. Journal of the National Cancer Institute. 2008;100:1771-1779.

Copyright © 2010 CancerConsultants. All Rights Reserved.

Tags: Uncategorized

You must be logged-in to the site to post a comment.