Acromegaly

Excerpt

Acromegaly is a rare condition with an approximate incidence of 3-11 new cases per million of population per year and a prevalence of approximately 60 per million (1). There are approximately 3000 identified individuals in the UK and 15000 in the USA, although it is possible that more cases exist but do not come to clinical attention. More recent studies suggest a higher incidence of acromegaly, up to 6.9 per 100,000 according to Italian data and 7.7 patients per million per year in Iceland (2,3). The condition was named by Pierre Marie in 1886 using the Greek words akron- extremities and megas- large to describe the typical clinical appearance of the condition (4).The disease occurs as a result of excessive secretion of growth hormone. In more than 99% of cases this is due to a benign pituitary growth hormone secreting adenoma. Pituitary carcinomas are exceedingly rare. Extremely infrequently acromegaly occurs as a result of ectopic secretion of growth hormone releasing hormone (GHRH) from a peripheral neuroendocrine tumor (5,6), excessive hypothalamic GHRH secretion (7), or can result after long term exogenous GH abuse (8). Approximately 5% of cases are associated with familial syndromes, most commonly multiple endocrine neoplasia type 1 (MEN1) syndrome, but also McCune Albright syndrome, familial acromegaly, Carney syndrome, and Familial Isolated Pituitary Adenoma (FIPA). Both genders are equally affected and the diagnosis is typically made in adults aged 40-60 years of age. Younger patients often have more aggressive disease due to more rapidly growing adenomas. Acromegaly is associated with multiple systemic complications and a higher risk of mortality if untreated. Very often a multi-modal treatment approach is required to manage the condition, including surgery, radiotherapy, somatostatin analogues, GH receptor antagonist, and dopamine agonist. The management should be individualized to the patient using best practice guidelines, clinical experience, and individual patient circumstances and guided by biomarkers and clinical predictors. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text, WWW.ENDOTEXT.ORG.