Acromegaly - evolving strategies.

Abstract

Growth hormone (GH)-cell adenomas are benign pituitary tumors which present with chronic high GH output. Hereditary GH-cell adenomas are rare and include MEN I, McCune Albright Syndrome, Carney complex and familial acromegaly. Most of the tumors causing acromegaly are sporadic. Acromegaly is a disfiguring and disabling disease and, if untreated, life expectancy is reduced by a decade. Elevated GH levels, hypertension and heart disease are major negative survival determinants in these patients. Current treatments for acromegaly attempt to control the disease by reducing growth hormone secretion from the tumor either by surgery, radiotherapy or medical therapy. The choice of therapy depends on age, general health, the severity and complications of the disease and dangers associated with each treatment. Assessment of disease activity in patients with acromegaly following treatment is a problem because no sensitive clinical parameters are available and there is no well-defined clinical endpoint that defines cure. Cure in acromegaly has been defined therefore as normalization of biochemical parameters. A consensus publication recommended biochemical cure be considered as nadir GH of less than 1 microm g/L after OGTT and a normal circulating IGF-I. In optimizing the control of acromegaly new therapeutic strategies are evolving (growth hormone receptor antagonist - pegvisomant, potent dopamine agonists, universal somatostatin receptor ligands, chimeric molecules). The aim of the evolving therapeutic strategies is to produce a normal life expectancy in patients with acromegaly.