Adrenal cortical tumours: epidemiological and familial aspects.

Abstract

Epidemiological data on the 14 cases of adrenal cortical tumour registered with the Manchester Children's Tumour Registry from 1954 and 1985 are presented. The incidence of adrenal cortical carcinomas was 0.3%, mainly in girls, most of whom presented with virilisation. The incidence of neoplastic disease among close relatives was ascertained, but, except in siblings, this was not significantly higher than would be expected. Evidence from extended pedigrees, however, indicates that at least four of the children could be members of families with the SBLA (sarcoma, breast and brain tumour, leukaemia, laryngeal and lung cancer, and adrenal cortical carcinoma) cancer family syndrome, and that other relatives may be at risk of developing such neoplasms.