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Subsequent Cancer Risk in Retinoblastoma Survivors

, by DCEG Staff

By 50 years after diagnosis with hereditary retinoblastoma, 33% of survivors develop a second cancer; 6% of survivors develop a third cancer.

Survivors of hereditary retinoblastoma have an increased long-term risk of subsequent epithelial cancers of certain sites and of developing multiple subsequent cancers, according to a study published on January 21, 2021, in the British Journal of Cancer

While advances in therapies have greatly improved survival rates following retinoblastoma (RB), a rare eye cancer typically diagnosed in early childhood, it is known that survivors of hereditary RB have a high risk of developing subsequent cancers, particularly sarcomas of the soft tissue and bone, as well as melanoma. The risks of other malignancies such as epithelial cancers and risk of developing more than one subsequent cancer are less well understood.

Using data from the Long-Term Follow-up Study of Retinoblastoma Survivors, Sara Schonfeld, Ph.D., staff scientist in the Radiation Epidemiology Branch, and colleagues, found that hereditary RB survivors had higher risks for subsequent epithelial cancers of the central nervous system, nasal cavity, oral cavity, and breast, but not of the kidney, bladder, uterus, pancreas, or lung, compared with the general population. They estimated that 33 percent of survivors of hereditary RB developed at least one malignancy within 50 years following RB diagnosis and six percent developed two or more, with much of the risk driven by sarcoma and melanoma. The authors did not find an increased risk of subsequent malignancies in survivors of nonhereditary RB.

This study advances our understanding of subsequent cancer risks in survivors of hereditary RB. Future investigations evaluating the site-specific risks associated with treatments for RB could aid long-term clinical management.


Schonfeld SJ et al. Long-term risk of subsequent cancer incidence among hereditary and nonhereditary retinoblastoma survivors. Br J Cancer. January 2021. DOI: 10.1038/s41416-020-01248-y. [Epub ahead of print]