Children with idiopathic hemihypertrophy and beckwith-wiedemann syndrome have different constitutional epigenotypes associated with wilms tumor.

Idiopathic hemihypertrophy (IH) is a congenital overgrowth syndrome associated with an increased risk of embryonal cancers in childhood. A related developmental disorder is Beckwith-Wiedemann syndrome (BWS), which increases risk for embryonal cancers, including Wilms tumor. Constitutional epigenetic alterations associated with BWS have been well characterized and include epigenetic alterations of imprinted genes on 11p15. The frequency of hypermethylation of H19 in children with IH and Wilms tumor, 20% (3/15), was significantly lower than the frequency in children with BWS and Wilms tumor, 79% (11/14; P = .0028). These results indicate that children with IH and Wilms tumor have different constitutional epigenotypes from those of children with BWS and Wilms tumor.

[1]  A. Feinberg,et al.  The history of cancer epigenetics , 2004, Nature Reviews Cancer.

[2]  Andrew P Feinberg,et al.  Epigenetic alterations of H19 and LIT1 distinguish patients with Beckwith-Wiedemann syndrome with cancer and birth defects. , 2002, American journal of human genetics.

[3]  A. Feinberg,et al.  Loss of imprinting of insulin-like growth factor-II (IGF2) gene in distinguishing specific biologic subtypes of Wilms tumor. , 2001, Journal of the National Cancer Institute.

[4]  P. Goodfellow,et al.  16q loss of heterozygosity and microsatellite instability in Wilms' tumor. , 2000, Journal of pediatric surgery.

[5]  A. Feinberg,et al.  Loss of imprinting in normal tissue of colorectal cancer patients with microsatellite instability , 1998, Nature Medicine.

[6]  H. Hoyme,et al.  Isolated hemihyperplasia (hemihypertrophy): report of a prospective multicenter study of the incidence of neoplasia and review. , 1998, American journal of medical genetics.

[7]  M. Tucker,et al.  Risk of cancer during the first four years of life in children from The Beckwith-Wiedemann Syndrome Registry. , 1998, The Journal of pediatrics.

[8]  P. Telzerow,et al.  Chromosome 11 uniparental isodisomy predisposing to embryonal neoplasms , 1991, The Lancet.

[9]  N. Breslow,et al.  Age distribution of Wilms' tumor: report from the National Wilms' Tumor Study. , 1988, Cancer research.

[10]  K. Kleinman,et al.  A nearly continuous measure of birth weight for gestational age using a United States national reference. , 2003, BMC pediatrics.