Pituitary gigantism: Causes and clinical characteristics

Abstract

Acromegaly and pituitary gigantism are very rare conditions resulting from excessive secretion of growth hormone (GH), usually by a pituitary adenoma. Pituitary gigantism occurs when GH excess overlaps with the period of rapid linear growth during childhood and adolescence. Until recently, its etiology and clinical characteristics have been poorly understood. Genetic and genomic causes have been identified in recent years that explain about half of cases of pituitary gigantism. We describe these recent discoveries and focus on some important settings in which gigantism can occur, including familial isolated pituitary adenomas (FIPA) and the newly described X-linked acrogigantism (X-LAG) syndrome.

Keywords: Adénomes hypophysaires familiaux isolés (FIPA); Aryl hydrocarbon receptor interacting protein gene; Familial isolated pituitary adenoma (FIPA); Gigantism; Gigantisme; Syndrome d’acrogigantisme lié au chromosome X (X-LAG); X-linked Acrogigantism (X-LAG) syndrome.