{"id":4422248,"date":"2025-01-11T09:54:03","date_gmt":"2025-01-11T15:54:03","guid":{"rendered":"https:\/\/myendoconsult.com\/learn\/topics\/acromegaly\/"},"modified":"2025-01-13T06:53:32","modified_gmt":"2025-01-13T12:53:32","slug":"acromegaly-notes","status":"publish","type":"oen_topic","link":"https:\/\/myendoconsult.com\/learn\/topics\/acromegaly-notes\/","title":{"rendered":"Acromegaly"},"content":{"rendered":"\n<h2 class=\"wp-block-heading\">ACROMEGALY<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Definition and Historical Background<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Definition<\/strong>: Chronic growth hormone (GH) excess from a GH-producing pituitary tumor results in the clinical syndrome of acromegaly.<\/li>\n\n\n\n<li><strong>Historical Note<\/strong>: Acromegaly was the first pituitary syndrome to be recognized, described by Pierre Marie in 1886.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Epidemiology<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Incidence<\/strong>: The annual incidence is estimated to be about three per 1 million persons in the general population.<\/li>\n\n\n\n<li><strong>Prevalence of GH-secreting Adenomas<\/strong>: A GH-secreting pituitary adenoma is the second most common hormone-secreting pituitary tumor.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Consequences of Untreated Acromegaly<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Increased Morbidity and Mortality<\/strong>: If untreated, this syndrome is associated with an increased risk of various complications and a higher mortality rate.<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">CLINICAL FEATURES<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Chronic GH Excess Effects<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong><a href=\"https:\/\/myendoconsult.com\/learn\/acromegaly\/\" data-type=\"link\" data-id=\"https:\/\/myendoconsult.com\/learn\/acromegaly\/\">Acral and Soft Tissue Overgrowth<\/a><\/strong><\/li>\n\n\n\n<li><strong>Progressive Dental Malocclusion (Underbite) and increased inter-dental distance<\/strong><\/li>\n\n\n\n<li><strong>Degenerative Arthritis<\/strong> due to overgrowth of chondral and synovial tissues in joints<\/li>\n\n\n\n<li><strong>Low-Pitched Sonorous Voice<\/strong><\/li>\n\n\n\n<li><strong>Headaches<\/strong><\/li>\n\n\n\n<li><strong>Malodorous Hyperhidrosis and Oily Skin<\/strong><\/li>\n\n\n\n<li><strong>Perineural Hypertrophy Leading to Nerve Entrapment<\/strong> (e.g., carpal tunnel syndrome)<\/li>\n\n\n\n<li><strong>Proximal Muscle Weakness<\/strong><\/li>\n\n\n\n<li><strong>Carbohydrate Intolerance<\/strong>: May initially present as <a href=\"https:\/\/myendoconsult.com\/learn\/diabetes-mellitus\/\" data-wpil-monitor-id=\"66\">diabetes mellitus<\/a><\/li>\n\n\n\n<li><strong>Hypertension<\/strong><\/li>\n\n\n\n<li><strong>Colonic Neoplasia<\/strong><\/li>\n\n\n\n<li><strong>Obstructive Sleep Apnea<\/strong><\/li>\n\n\n\n<li><strong>Cardiac Dysfunction<\/strong><\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Mass Effects of GH-Producing Pituitary Macroadenomas (&gt; 10 mm)<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Visual Field Defects<\/strong><\/li>\n\n\n\n<li><strong>Oculomotor Pareses<\/strong><\/li>\n\n\n\n<li><strong>Headaches<\/strong><\/li>\n\n\n\n<li><strong>Pituitary Insufficiency<\/strong><\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Characteristic Appearance<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Coarsening of Facial Features<\/strong><\/li>\n\n\n\n<li><strong>Prognathism (Jaw Protrusion)<\/strong><\/li>\n\n\n\n<li><strong>Frontal Bossing<\/strong><\/li>\n\n\n\n<li><strong>Spadelike Hands<\/strong><\/li>\n\n\n\n<li><strong>Wide Feet<\/strong><\/li>\n\n\n\n<li><strong>Progressive Increase in Shoe, Glove, Ring, or Hat Size<\/strong><\/li>\n\n\n\n<li>These changes may be subtle and develop slowly, often going unrecognized by the patient, family, or physician.<\/li>\n\n\n\n<li><strong>Diagnosis Delay<\/strong>: On average, there is an 8.5-year delay from the onset of the first symptoms to the eventual diagnosis.<\/li>\n\n\n\n<li><strong>Comparison with Earlier Photographs<\/strong>: Useful in confirming clinical suspicion.<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">DIAGNOSTIC CONSIDERATIONS<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">GH Levels and Their Limitations<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>High Plasma GH Not Always Diagnostic<\/strong>: Basal plasma GH levels can be elevated in:\n<ul class=\"wp-block-list\">\n<li>Poorly controlled <a href=\"https:\/\/myendoconsult.com\/learn\/glucose-bad-mnemonic\/\" data-wpil-monitor-id=\"67\">diabetes mellitus<\/a><\/li>\n\n\n\n<li>Chronic hepatic or renal failure<\/li>\n\n\n\n<li>Conditions with protein-calorie malnutrition (e.g., anorexia nervosa)<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Criteria for Acromegaly Diagnosis<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Lack of GH Suppression<\/strong>\n<ul class=\"wp-block-list\">\n<li><a href=\"https:\/\/myendoconsult.com\/learn\/acromegaly-testing\/\" data-type=\"link\" data-id=\"https:\/\/myendoconsult.com\/learn\/acromegaly-testing\/\">GH does not suppress to less than 0.4 ng\/dL<\/a> after an oral glucose load (75\u2013100 g). Learn about the <a href=\"https:\/\/myendoconsult.com\/learn\/growth-hormone-suppression-test\/\" data-type=\"link\" data-id=\"https:\/\/myendoconsult.com\/learn\/growth-hormone-suppression-test\/\">growth hormone suppression test<\/a> for acromegaly.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Elevated <a href=\"https:\/\/myendoconsult.com\/learn\/serum-igf-1-to-igfbp-3-molar-ratio\/\" data-wpil-monitor-id=\"68\">Serum IGF-1<\/a><\/strong>\n<ul class=\"wp-block-list\">\n<li>Increased serum concentration of insulin-like growth factor 1 (IGF-1), adjusted for age and gender.<\/li>\n\n\n\n<li>IGF-1 (formerly known as somatomedin C) is a GH-dependent growth factor responsible for many effects of GH.<\/li>\n\n\n\n<li>Serum IGF-1 levels are rarely falsely elevated.<\/li>\n\n\n\n<li><strong>Exception<\/strong>: During pregnancy, IGF-1 can rise two- to threefold above the upper limit of normal values.<\/li>\n<\/ul>\n<\/li>\n<\/ol>\n\n\n\n<h3 class=\"wp-block-heading\">Imaging and Additional Workups<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Magnetic Resonance Imaging (MRI) of the Pituitary<\/strong>\n<ul class=\"wp-block-list\">\n<li>Evaluate for adenoma and associated sellar or suprasellar extension.<\/li>\n\n\n\n<li><strong>MRI (Coronal View)<\/strong>: May show a pituitary macroadenoma eroding the sellar floor, extending into the cavernous sinus, and up to the optic chiasm.<\/li>\n\n\n\n<li><strong>MRI (Midline Sagittal View)<\/strong>: May show extension of the macroadenoma into the sphenoid sinus and suprasellar region.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Visual Field Examination<\/strong>\n<ul class=\"wp-block-list\">\n<li>Quantitative perimetry to assess for bitemporal hemianopsia or other visual defects.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>If No Adenoma Is Detected on Pituitary Imaging<\/strong>\n<ul class=\"wp-block-list\">\n<li>Measure plasma GH-releasing hormone (GHRH) concentration.<\/li>\n\n\n\n<li>Perform CT of the chest and abdomen to search for an ectopic GHRH-producing tumor (e.g., pancreatic or small cell lung neoplasm).<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">TREATMENT<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Indications and Goals<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Who Needs Treatment?<\/strong>: All patients with acromegaly.<\/li>\n\n\n\n<li><strong>Main Goals<\/strong>:\n<ol class=\"wp-block-list\">\n<li>Prevent long-term consequences of GH excess.<\/li>\n\n\n\n<li>Remove the sellar mass.<\/li>\n\n\n\n<li>Preserve normal pituitary tissue and function.<\/li>\n<\/ol>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Treatment Options<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Surgery<\/strong>\n<ul class=\"wp-block-list\">\n<li><strong>Transsphenoidal Adenectomy<\/strong>: Performed by an experienced neurosurgeon.<\/li>\n\n\n\n<li>Treatment of choice for acromegaly.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Targeted Irradiation<\/strong>\n<ul class=\"wp-block-list\">\n<li><strong>Gamma Knife Radiotherapy<\/strong> may be used if necessary.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong><a href=\"https:\/\/myendoconsult.com\/learn\/acromegaly-medications-mechanism-of-action\/\" data-type=\"link\" data-id=\"https:\/\/myendoconsult.com\/learn\/acromegaly-medications-mechanism-of-action\/\">Medical Therapy<\/a><\/strong>\n<ul class=\"wp-block-list\">\n<li>Often used as a supplement or if surgery and\/or radiotherapy are contraindicated or incomplete in their control of GH levels. Examples of medical therapy include <a href=\"https:\/\/myendoconsult.com\/learn\/somatostatin-analogs-and-acromegaly\/\" data-type=\"link\" data-id=\"https:\/\/myendoconsult.com\/learn\/somatostatin-analogs-and-acromegaly\/\">somatostatin analogs<\/a> and <a href=\"https:\/\/myendoconsult.com\/learn\/pegvisomant-for-acromegaly\/\" data-type=\"link\" data-id=\"https:\/\/myendoconsult.com\/learn\/pegvisomant-for-acromegaly\/\">pegvisomant<\/a>.<\/li>\n<\/ul>\n<\/li>\n<\/ol>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">POST-TREATMENT CHANGES<\/h2>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Regression of Soft Tissue Excess<\/strong>\n<ul class=\"wp-block-list\">\n<li>Marked regression after successful surgical treatment.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Permanent Bone Changes<\/strong>\n<ul class=\"wp-block-list\">\n<li>Bone deformities (e.g., enlarged jaw, frontal bossing) do not fully revert.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Possible Need for Combined Oral and Plastic Surgery<\/strong>\n<ul class=\"wp-block-list\">\n<li>Mandibular osteotomies<\/li>\n\n\n\n<li>Recession of the supraorbital ridges<\/li>\n\n\n\n<li>Rhinoplasties<\/li>\n\n\n\n<li>Reduction of tongue size<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Hypertrophic Osteoarthropathy<\/strong>\n<ul class=\"wp-block-list\">\n<li>Disabling changes, especially in the hip or other large joints, may require joint replacement.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">FOLLOW-UP AND SURVEILLANCE<\/h2>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Colorectal Screening<\/strong>\n<ul class=\"wp-block-list\">\n<li>Patients with acromegaly have an increased risk of colorectal adenomas and cancer.<\/li>\n\n\n\n<li>Regular colonoscopic screening is advised.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n","protected":false},"excerpt":{"rendered":"<p>ACROMEGALY Definition and Historical Background Epidemiology Consequences of Untreated Acromegaly CLINICAL FEATURES Chronic GH Excess Effects Mass Effects of GH-Producing Pituitary Macroadenomas (&gt; 10 mm) Characteristic Appearance DIAGNOSTIC CONSIDERATIONS GH Levels and Their Limitations Criteria for Acromegaly Diagnosis Imaging and Additional Workups TREATMENT Indications and Goals Treatment Options POST-TREATMENT CHANGES FOLLOW-UP AND SURVEILLANCE<\/p>\n","protected":false},"featured_media":0,"template":"","oen_topic_chapter":[682],"class_list":["post-4422248","oen_topic","type-oen_topic","status-publish","hentry","oen_topic_chapter-pituitary-gland","post-wrapper","thrv_wrapper"],"_links":{"self":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422248","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic"}],"about":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/types\/oen_topic"}],"version-history":[{"count":8,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422248\/revisions"}],"predecessor-version":[{"id":4422265,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422248\/revisions\/4422265"}],"wp:attachment":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/media?parent=4422248"}],"wp:term":[{"taxonomy":"oen_topic_chapter","embeddable":true,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic_chapter?post=4422248"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}