Retinoblastoma survivors often die from other malignancies

NEW YORK (Reuters Health) - Survivors of hereditary retinoblastoma are 35 times more likely to die from cancer than are people in the general population, according to a report in the Journal of the National Cancer Institute for April 15. For survivors of non-hereditary retinoblastoma, the risk is increased 2.5-fold.

Prior research has shown that subsequent cancers are common among survivors of hereditary retinoblastoma, but data from long-term survivors who were treated with radiotherapy is limited, lead author Dr. Chu-Ling Yu, from the National Cancer Institute, Rockville, Maryland, and colleagues explain.

In the current analysis, the researchers examined cause-specific mortality among 1854 patients who were survivors of retinoblastoma diagnosed between 1914 and 1996 at two centers.

A total of 151 cancer-related deaths were recorded among the 1092 survivors of hereditary retinoblastoma. Twelve such deaths were noted among the remaining 762 patients who survived non-hereditary retinoblastoma.

Malignancies often seen in survivors included sarcomas, melanomas, and cancers of the central nervous system. The elevated risk of a cancer-related death extended for more than 40 years after the retinoblastoma diagnosis.

Commenting on an earlier analysis they conducted, the authors note that the 13 additional years of follow-up in the present analysis uncovered a link between retinoblastoma survival and mortality from cancers of the corpus uteri, mostly sarcomas.

Consistent with previous reports, subjects treated with radiotherapy were at greater risk for developing cancer than those who did not receive radiation.

At 50 years after diagnosis of retinoblastoma, the cumulative mortality from subsequent cancers was 25.5% for hereditary retinoblastoma and 1.0% for nonhereditary retinoblastoma.

By contrast, survivors of both types of retinoblastoma were not at increased risk for non-neoplastic causes of death.

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

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