Adrenal Cortical Hyperplasia

Abstract

Adrenal cortical hyperplasia is characterized by an increase in cortical cells, which usually occurs bilaterally. Adrenal cortical hyperplasia generally affects both adrenal glands and may be diffuse or nodular or show features of both patterns. Adrenal glands may show hyperplasia primarily and secondarily. Primary adrenocorticotropic hormone (ACTH)-independent cortical hyperplasia is seen sporadically or in Carney complex. ACTH-dependent cortical hyperplasia occurs in congenital adrenal hyperplasia (adrenogenital syndrome), an autosomal recessive defect in an enzymatic step of steroid synthesis (most commonly 21-hydroxylase). The adrenal glands are enlarged as the result of continuous stimulation of the adrenal cortex by ACTH. Congenital adrenal hyperplasia is the most common cause of primary adrenal insufficiency in children. Secondary adrenal cortical hyperplasia is caused by pituitary ACTH adenoma, often a microadenoma, or ectopic ACTH production. The adrenal glands show a diffuse and/or micronodular pattern. These specimens, however, rarely are removed and evaluated by pathologists, because removal of the pituitary tumor is the mainstay of treatment. Other ectopic sites of ACTH production may occur and may be associated with hypercortisolism.