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Patterns of Cause-Specific Mortality in Survivors of Retinoblastoma, 1914-2016

, by DCEG Staff

Mortality patterns among adult survivors of retinoblastoma differ by hereditary status, cause of death, calendar year of diagnosis, treatment, and time since diagnosis, according to a study published January 30, 2019, in the Journal of the National Cancer Institute.

For the past 30 years, investigators at the National Cancer Institute, Memorial Sloan Kettering Cancer Center, and University of Massachusetts Medical Center, have followed a population of retinoblastoma survivors, a rare cancerous tumor of the eye that occurs in childhood, documenting their risk for subsequent health outcomes. The cohort includes survivors of hereditary and non-hereditary disease. Previous work from the study has demonstrated elevated risk of death among survivors of hereditary retinoblastoma, particularly from subsequent bone cancer, soft tissue sarcoma, and melanoma.

In this latest report, the investigators present new data from an extended follow-up through over six decades of life for some survivors. Most deaths occurred among the 1,129 hereditary retinoblastoma survivors, who had more than an 8-fold increased overall risk of death compared with the general population. The highest risks were for deaths due to subsequent malignancies, which persisted through the entire duration of follow-up, highlighting the need to develop long-term clinical management guidelines for hereditary retinoblastoma survivors. Elevated risk for death due to cancers in the pancreas, large intestine, and kidney were noted for the first time. 

The study also provided some reassuring news for retinoblastoma survivors treated in the past. Despite the high risks of death due to subsequent malignancies, hereditary retinoblastoma survivors did not have significantly elevated risk of death due to non-cancer causes, and for survivors of non-hereditary retinoblastoma, long-term mortality was largely comparable to rates observed in the general population, overall and for both cancer- and non-cancer-related deaths.

Reference:

Kleinerman RA, Tucker MA, Morton LM, et al. Patterns of Cause-Specific Mortality Among 2053 Survivors of Retinoblastoma, 1914–2016J Natl Cancer Inst, January 30, 2019. DOI: 10.1093/jnci/djy227 [Epub ahead of print]