GENERATION OF HOMOZYGOSITY AT THE C-HA-RAS-1 LOCUS ON CHROMOSOME-11P IN AN ADRENAL ADENOMA FROM AN ADULT WITH WIEDEMANN-BECKWITH SYNDROME

Abstract

Generation of homozygosity for the human c-Ha-ras-1 locus on the short arm of chromosome #11 (11p) has been demonstrated for an adrenal adenoma from an adult with Wiedemann-Beckwith syndrome (WBS). This is the first demonstration of loss of somatic heterozygosity for a locus on 11p in an adrenal neoplasm and is the first instance where a tumor of any type, from a patient with WBS, shows loss of heterozygosity in this region of the genome. Generation of homozygosity in an adenoma, rather than a carcinoma, demonstrates that this mechanism is an early event in tumorigenesis rather than a late event associated with tumor progression.