title: Expression of a high molecular weight form of insulin-like growth factor II in a Beckwith-Wiedemann syndrome associated adrenocortical adenoma.
abstract: Beckwith-Wiedemann syndrome is a rare condition (1/13,700 live births) occurring in both inherited and sporadic forms in the population. It is manifest as a fetal overgrowth syndrome, in which hypertrophy dominates the clinical picture. An additional complication is that these children are predisposed to a specific subset of childhood neoplasms, amongst which are Wilms' tumour and adrenocortical carcinoma. We report here the synthesis by an associated adrenal tumour of large quantities of a high molecular weight form of insulin-like growth factor II (IGF-II), associated with profound suppression of circulating IGFs in the patient's serum. As with other tumours of this type, the tumours showed loss of material on chromosome 11p.
authors.affiliation: Department of Anatomy, University of Cambridge, UK.
authors.forename: P N
authors.initials: PN
authors.lastname: Schofield
authors.forename: A
authors.initials: A
authors.lastname: Nystrom
authors.forename: J
authors.initials: J
authors.lastname: Smith
authors.forename: L
authors.initials: L
authors.lastname: Spitz
authors.forename: D
authors.initials: D
authors.lastname: Grant
authors.forename: J
authors.initials: J
authors.lastname: Zapf
mesh_terms: D000236:Adenoma / Q000209:etiology / Q000235:genetics / Q000378:metabolism*; D000306:Adrenal Cortex Neoplasms / Q000209:etiology / Q000235:genetics / Q000378:metabolism*; D001506:Beckwith-Wiedemann Syndrome / Q000150:complications / Q000378:metabolism*; D005838:Genotype; D006801:Humans; D007223:Infant; D007335:Insulin-Like Growth Factor II / Q000737:chemistry / Q000378:metabolism*; D008297:Male; D008970:Molecular Weight; D009363:Neoplasm Proteins / Q000737:chemistry / Q000378:metabolism*
chemical_list: D009363:Neoplasm Proteins; D007335:Insulin-Like Growth Factor II