Long-term retinoblastoma survivors are at a greater risk of dying from subsequent cancers and this elevated risk extends beyond 40 years, according to the results of a study published in the Journal of the National Cancer Institute.1

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.

Although retinoblastoma is a curable disease, it has been shown that survivors of hereditary retinoblastoma have a significantly higher risk of developing subsequent cancers when compared to the general population. Some survivors develop more than three subsequent cancers.

As more long-term follow-up data becomes available, researchers have begun to evaluate not only the risk of developing subsequent cancers, but also the risk of dying from these cancers. In addition, the research is not limited to hereditary retinoblastoma survivors, but all survivors and especially those treated with radiation.

Researchers affiliated with the National Cancer Institute evaluated a cohort of 1,854 retinoblastoma survivors by examining medical records from two medical centers in New York and Boston. Among 1,092 hereditary retinoblastoma survivors, there were 151 deaths from subsequent cancers compared with 12 deaths among 762 nonhereditary retinoblastoma survivors. The researchers found that the risk of death from subsequent cancers extended beyond 40 years in hereditary retinoblastoma survivors. Among both hereditary and nonhereditary retinoblastoma survivors, the rates of death from subsequent cancers were higher among those who had been treated with radiation. The most common subsequent cancers were sarcomas, melanomas, and cancers of the brain and other parts of the nervous system.

The researchers concluded that retinoblastoma survivors are at no greater risk than the general population of dying from non-cancerous causes; however, they are at a much greater risk of dying from subsequent cancers. They suggest that this elevated risk should influence screening patterns for the early detection and treatment of subsequent cancers in this group of survivors.

Reference:

1 Yu CL, Tucker MA, Abramson DH, et al. Cause-specific mortality in long-term survivors of retinoblastoma. Journal of the National Cancer Institute. 2009; 101: 581-591.

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