Hereditary Retinoblastoma and Risk of Lung Cancer

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Abstract
Patients who have survived hereditary retinoblastoma are at increased risk of dying of a sarcoma, a melanoma, or a brain tumor. The increased risk has been attributed to germline mutations in the retinoblastoma-1 (RB1) gene, which encodes the cell cycle regulatory protein pRb (1). However, the extent to which patients with hereditary retinoblastoma are at increased risk of dying of common epithelial cancers of adulthood, such as lung cancer, is unknown. Somatic mutations in the RB1 gene are known to contribute to the development of lung cancer (2,3), and evidence of increased risk of lung cancer in adult RB1 mutation carriers has been reported (4,5), but questions remain about the association between hereditary retinoblastoma and lung cancer risk (6). In our earlier follow-up studies of 1604 one-year survivors of retinoblastoma (1,7) followed for a median of 17 years, we did not detect an increased risk of lung cancer, but few patients had reached 40 years of age, when the incidence rates for most epithelial cancers begin to rise. After 7 additional years of follow-up, we have again assessed these patients' risk of dying of lung cancer.

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