Chapter 12 - Medical management of pituitary gigantism and acromegaly

Abstract

Acromegaly and pituitary gigantism are two related clinical manifestations of chronic growth hormone (GH) and insulin-like growth factor 1 (IGF-1) hypersecretion, usually from a pituitary adenoma. Management of acromegaly is guided by consensus reports based on accumulated clinical trials, in which medical therapies including somatostatin analogs, the GH receptor antagonist pegvisomant, and dopamine agonists play a key role alongside neurosurgery. Pituitary gigantism represents a severe subtype of acromegaly, as it affects children/adolescents whose growth plates have not fused. Apart from the risk of increased final adult height, patients with pituitary gigantism also have relatively aggressive and treatment resistant disease. This phenotype is driven by the presence of genetic mutations in nearly 50% of pituitary gigantism cases, including AIP mutations, X-linked acrogigantism syndrome, and McCune–Albright syndrome. This chapter assesses the consensus medical therapy of acromegaly and highlights specific aims and challenges for the effective management of patients with pituitary gigantism.