title: Congenital abnormalities and hepatoblastoma: a report from the Children's Oncology Group (COG) and the Utah Population Database (UPDB).
abstract: Beckwith-Wiedemann Syndrome (BWS) and familial adenomatous polyposis (FAP) are known to predispose to hepatoblastoma (HB). A case-control study was conducted through the Children's Oncology Group (COG) to study the association of HB with isolated congenital abnormalities. Cases (N = 383) were diagnosed between 2000 and 2008. Controls (N = 387) were recruited from state birth registries, frequency matched for sex, region, year of birth, and birth weight. Data on congenital abnormalities among subjects and covariates were obtained by maternal telephone interview. Odds ratios (OR) and 95% confidence intervals (CI) describing the association between congenital abnormalities with HB, adjusted for sex, birth weight, maternal age and maternal education, were calculated using unconditional logistic regression. There was a significant association of HB with kidney, bladder, or sex organ abnormalities (OR = 4.75; 95% CI: 1.74-13) which appeared to be specific to kidney/bladder defects (OR = 4.3; 95% CI: 1.2-15.3) but not those of sex organs (OR = 1.24; 95% CI: 0.37-4.1). Elevated but non-significant ORs were found for spina bifida or other spinal defects (OR = 2.12; 95% CI: 0.39-11.7), large or multiple birthmarks (OR = 1.33; 95% CI: 0.81-2.21). The results were validated through the Utah Population Database (UPDB), a statewide population-based registry linking birth certificates, medical records, and cancer diagnoses. In the UPDB, there were 29 cases and 290 population controls matched 10:1 on sex and birth year. Consistent with the COG findings, kidney/bladder defects were associated with hepatoblastoma. These results confirm the association of HB with kidney/bladder abnormalities.
authors.affiliation: Division of Hematology/Oncology, Children's Hospital Los Angeles, Los Angeles, California; Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, California.
authors.forename: Rajkumar
authors.initials: R
authors.lastname: Venkatramani
authors.forename: Logan G
authors.initials: LG
authors.lastname: Spector
authors.forename: Michael
authors.initials: M
authors.lastname: Georgieff
authors.forename: Gail
authors.initials: G
authors.lastname: Tomlinson
authors.forename: Mark
authors.initials: M
authors.lastname: Krailo
authors.forename: Marcio
authors.initials: M
authors.lastname: Malogolowkin
authors.forename: Wendy
authors.initials: W
authors.lastname: Kohlmann
authors.forename: Karen
authors.initials: K
authors.lastname: Curtin
authors.forename: Rachel K
authors.initials: RK
authors.lastname: Fonstad
authors.forename: Joshua D
authors.initials: JD
authors.lastname: Schiffman
mesh_terms: D000328:Adult; D002648:Child; D015331:Cohort Studies; D000013:Congenital Abnormalities / Q000473:pathology*; D003710:Demography; D005260:Female; D018197:Hepatoblastoma / Q000150:complications*; D006801:Humans; D008297:Male; D012042:Registries; D015203:Reproducibility of Results; D058028:Research Report; D014589:Utah; D055815:Young Adult
keywords: Beckwith-Wiedemann syndrome; case-control study; congenital abnormality; congenital anomaly; familial adenomatous polyposis; hepatoblastoma; kidney abnormalities