Risk of cancer during the first four years of life in children from The Beckwith-Wiedemann Syndrome Registry,☆☆,,★★

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Abstract

To determine the incidence and relative risk (RR) of cancer in children with Beckwith-Wiedemann syndrome (BWS), children with BWS were followed up from birth until death, diagnosis of cancer, fourth birthday, or last day of follow-up. A total of 183 children with BWS were followed up for 482 person-years. The end points were incidence of cancer, RR of cancer, and RR associated with specific BWS phenotypic features. Thirteen children were identified with cancers before the fourth year of life in comparison with fewer than one cancer expected in this group on the basis of general population rates over the same period. The average annual incidence of cancer in the first 4 years of life was 0.027 cancer per person-year. The RR of Wilms tumor (RR = 816; 95% confidence interval [CI], 359-1156), neuroblastoma (RR = 197; 95% CI, 22-711), and hepatoblastoma (RR = 2280; 95% CI, 928-11,656) were statistically significant. Asymmetry of the limbs (hemihypertrophy) was the only clinical feature associated with an increased RR of cancer (RR = 4.6; 95% CI, 1.5-14.2). Given the high incidence of cancer in infancy and early childhood of patients with BWS, a prospective study is warranted to address the utility of screening for cancer. (J Pediatr 1998;132:398-400)

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Methods

Between April 1989 and January 1994, 192 patients with BWS were registered in the BWS parent support group. Parents contacted the support group for a variety of reasons including seeking information on BWS, identifying other families with a child who has BWS, and seeking referrals to pediatric specialists. As part of the initial registration for the support group, parents were interviewed and asked a series of questions regarding the cardinal features of BWS.5 With regard to informed consent,

Results

We followed up 183 patients for 482 patient-years during the first 4 years of life. The mean age of the cohort was 3.7 years, and the mean age at which the patient was signed up for The BWS Network was 1.6 years; 57% were boys. Thirteen children had cancer (six had WT, five had hepatoblastoma, and two had neuroblastoma) within the first 4 years of life. An additional patient, not included in the cohort, was diagnosed at 65 months. The median and mean ages at the time of the cancer diagnosis

Discussion

The association of early childhood cancer and BWS is well established, but the magnitude of this risk during infancy and early childhood is not. Previous retrospective analyses of a series of children with BWS focused solely on the frequency of cancer, ranging from 4%5 to 7.5%.1 No study has identified a cohort of children with BWS to determine the incidence of cancer. Our results indicate that both the average incidence and RR of cancer in the first 4 years of life are elevated with

Acknowledgements

We thank the participating families who made the project possible; the staff of the Genetic Epidemiology Branch at the National Cancer Institute for their helpful comments and encouragement; and the staff of Westat Inc. and IMS for their support.

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From Genetic Epidemiology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland; and Division of Pediatric Hematology-Oncology, Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri.

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Reprint requests: Michael R. DeBaun, EPN 400, Genetic Epidemiology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892-7372.

Dr. DeBaun is currently funded by the Robert Woods Johnson Minority Faculty Development Award.

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