Mini-symposium: Endocrine pathologySyndromes associated with abnormalities in the adrenal cortex

Recognizing that adrenal pathology is a component of many genetic syndromes is important for clinical management and genetic counselling. These syndromes may be divided into those which cause excessive adrenal growth including tumours, and those which cause hypoplasia or destruction of the adrenal glands. Syndromes associated with hyperplasia and tumour development include Beckwith-Wiedemann, Li-Fraumeni, Carney complex, McCune-Albright, multiple endocrine neoplasia type 1, hereditary nonpolyposis colorectal carcinoma, familial adenomatous polyposis, primary bilateral macronodular adrenal hyperplasia, and congenital adrenal hyperplasia. Syndromes associated with adrenal dysgenesis or atrophy include X-linked adrenal hypoplasia, IMAGe syndrome, MIRAGE syndrome, familial glucocorticoid deficiency, X-linked adrenoleucodystrophy, Allgrove syndrome, Wolman syndrome, autoimmune polyendocrine syndrome and steroid resistant nephrotic syndrome. The clinical, genetic, and histopathological features of each disease will be discussed.