Risk of cancer during the first four years of life in children from The Beckwith-Wiedemann Syndrome Registry☆,☆☆,★,★★
Section snippets
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.
References (14)
- et al.
Complete and incomplete forms of Beckwith-Wiedemann syndrome: their oncogenic potential
J Pediatr
(1980) Wilms' tumor, overgrowth, and fetal growth factors: a hypothesis
Cancer Genet Cytogenet
(1986)- et al.
Hematologic abnormalities in Fanconi anemia: an International Fanconi Anemia Registry study
Blood
(1994) - et al.
Clinical features and natural history of Beckwith-Wiedemann syndrome: presentation of 74 new cases
Clin Genet
(1994) Tumor and hemihypertrophy associated with Wiedemann-Beckwith's syndrome [letter]
Eur J Pediatr
(1983)- et al.
Screening for the early detection of cancer
Cancer Invest
(1986) - et al.
Wiedemann-Beckwith syndrome: presentation of clinical and cytogenetic data on 22 new cases and review of the literature
Hum Genet
(1986)
Cited by (378)
Precision Oncology in Pediatric Cancer Surgery
2024, Surgical Oncology Clinics of North AmericaPediatric Upper Abdominal Masses: Current Practical Imaging Assessment
2024, Seminars in RoentgenologyA Case report: Co-occurrence of IMAGe syndrome and Rhabdomyosarcoma
2021, Cancer GeneticsBeckwith-Widemann Macroglossia: The Role of Surgical Tongue Reduction
2024, Cleft Palate Craniofacial JournalHepatoblastoma: A Review
2023, SSRNPediatric liver cancer
2023, Chronic Disease and Disability: The Pediatric Gastrointestinal Tract, Second Edition. Medical and Surgical Perspectives including Infection and Pain
- ☆
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.
- ☆☆
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.
- ★★
9/21/85587