Neurosurg Focus 29 (4):Introduction, 2010 Introduction Acromegaly: a fascinating pituitary common problem is osteoarthritis of the large joints, of- ten requiring surgical procedures. Cardiovascular effects disorder include hypertension, cardiomegaly, and heart failure; pulmonary consequences include obstructive sleep ap- nea (an effect of soft-tissue enlargement of the posterior MARY LEE VANCE , M.D. pharynx and also central sleep apnea). Gastrointestinal tract effects include increased prevalence of colon polyps Department of Medicine, Division of Endocrinology and Me ­ and potentially colon cancer. Metabolic effects of exces- tab­­olism, University of Virginia Health System, Charlottesville, Vir­­ginia sive growth hormone are insulin resistance and diabetes mellitus (in 25%–50% of patients). In women, infertility or loss of regular menses may occur. Men often have a Although acromegaly and gigantism are uncom- subnormal testosterone level. There is also an increased mon, since the clinical features originally reported by Jo- prevalence of thyroid gland nodules and ovarian cysts. hannes Wier in 1567 and Pierre Marie in 1886, there has Another effect of excessive growth hormone production been considerable interest in description of the condition, is reduction in fat mass and increase in muscle mass. elucidating the etiology and pathophysiology as well as A continuing problem is the delay in diagnosis, often the development of treatments. There is a long-standing 10 years or more from the onset of symptoms. Patients history of giants in the circus, skeletons of giants on dis- are treated for the acromegaly-related consequences such play, and more recently, giants playing college and pro- as hypertension, heart disease, sleep apnea, diabetes mel- fessional basketball. litus, and osteoarthritis without recognition of the under- Acromegaly (along with gigantism) is a systemic dis- lying etiology. The gradual change in physical features ease resulting from a growth hormone–secreting pituitary and the lack of recognition of the physical characteristics, adenoma and, rarely, from an ectopic tumor (pancreatic which patients often attribute to aging, result in the delay or carcinoid tumor) producing growth hormone–releas- in diagnosis. ing hormone. Although the clinical features, such as fa- Acromegaly includes 2 general features that are the cial deformities including frontal bossing, prognathism, focus of treatment: the tumor and the excess growth hor- large hands and feet (and gigantism), are recognizable, mone and its consequences. This issue of Neurosurgical the less commonly appreciated features are the risk of Focus describes the history of recognition of acromegaly, premature death and significant morbidity if the disease the pathogenesis of the disease, changes in cranial anat- is not treated successfully. omy, the specific features of growth hormone–secreting Acromegaly is a systemic disease that affects the tumors, types of treatment (surgery, radiation therapy, whole body including thickened skin, skin tags, hyper- medical treatments), and the outcomes of these treat- hidrosis, and cystic acne. Skeletal effects include frontal ments. Because of the delay in diagnosis, many patients bossing, sinus enlargement (resonate voice), enlargement harbor a large, invasive, pituitary adenoma when the dis- of the lower jaw (“alligator jaw”) with dental malocclusion ease is finally recognized. Resection is the first treatment and increased spacing between the teeth, enlargement of but patients often require additional therapies for a sus- the larynx (deepening of the voice), enlargement of the tained remission with the goal of reducing morbidity and bones of the hands and feet, carpal tunnel syndrome, and the risk of premature death. Patients with acromegaly enlargement of the rib cage (“barrel chest”). Bone density and other types of pituitary adenomas exemplify the need is usually normal but may be reduced by concomitant hy- for multimodality therapy to achieve optimal outcomes. pogonadism. As a result of bony overgrowth, the most (DOI: 10.3171/2010.10.FOCUS.Intro) Neurosurg Focus / Volume 29 / October 2010 1 Unauthenticated | Downloaded 09/26/21 12:06 AM UTC.