Survival after retinoblastoma: long-term consequences and family history of cancer.
Retinoblastoma (Rb) is a rare childhood tumor of the eye. In the heritable form, tumors are often bilateral and survivors have a greatly increased risk both for a second malignancy and for having children with Rb. Familial patterns of both cancer and birth defects are poorly understood in families with a heritable cancer, and little is known of the ways that a heritable cancer affects the lives of long-term survivors. To find out more about these and other issues in the lives of long-term survivors of childhood and adolescent cancer, we interviewed 56 adult survivors of retinoblastoma (15 with the heritable form) and 84 brothers and sisters as controls, who formed part of a large retrospective cohort study. Rb survivors were interviewed between 1980 and 1983, when they were 30 years old on average. Types of employment and health problems did not differ between survivors and controls, regardless of sight, but the income of blind survivors was considerably less than that of partially sighted survivors. Despite similar marriage rates, fewer survivors than controls reported a pregnancy (RR = 0.45; 95% CI; 0.24-0.83 for both sexes combined). Parents of children with heritable Rb seemed more likely to have had cancer than parents in families with nonheritable Rb (P = 0.06), and mothers were more likely than fathers to be affected (P = 0.01). This small series suggests that having retinoblastoma may have many long-term consequences, reaching beyond genetic and physical effects to touch family life and income attainment and the health of other family members. Follow-up of more modern cohorts and the use of molecular tools will clarify the long-term consequences of more recent therapies, and patterns of familiar cancer.
Byrne
J
J
Epidemiology and Biostatistics Program of the National Cancer Institute, National Institutes of Health, Bethesda, Maryland.
Fears
T R
TR
Whitney
C
C
Parry
D M
DM
D000124:Achievement; D000328:Adult; D015331:Cohort Studies; D000013:Congenital Abnormalities / Q000453:epidemiology; D005134:Eye Neoplasms / Q000235:genetics* / Q000401:mortality*; D005192:Family Health; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007182:Income; D008297:Male; D008393:Marriage; D009369:Neoplasms / Q000453:epidemiology; D011247:Pregnancy; D011379:Prognosis; D012175:Retinoblastoma / Q000235:genetics* / Q000401:mortality*; D012189:Retrospective Studies; D017741:Survivors