Acromegaly (Eosinophilic Adenoma Syndrome)

Acromegaly facial features[1]

Acromegaly is characterized by excessive secretion of growth hormone, usually caused by a somatotroph pituitary adenoma.

Presentation

The diagnosis of acromegaly is frequently delayed due to the slow progression of signs and symptoms. Patients with large tumors may experience symptoms due to the local mass effects of the pituitary lesion. These include headaches, visual abnormalities, typically bitemporal hemianopsia, and cranial nerve palsies. Hypersecretion of prolactin occurs if its inhibitory feedback from the hypothalamus is disturbed. Pituitary adenomas may also result in decreased secretion of other hormones by the pituitary gland.

Oversecretion of GH and IGF-1 causes acral and soft tissue overgrowth with skin thickening and organomegaly leading to arthritis, abnormal development of facial features, diabetes mellitus, hypertension and other cardiovascular diseases, carpal tunnel syndrome, obstructive sleep apnea, and various other symptoms. Patients present with increased foot size due to abnormal development of feet and increased ring size due to excessive fingers growth. Hyperhidrosis may also occur. Increased urinary secretion of calcium and phosphate is also associated with acromegaly.

Jaw & Teeth
  • Macroglossia Symptoms due to excess of GH/IGF-I These include the following: Soft tissue swelling and enlargement of extremities Increase in ring and/or shoe size Hyperhidrosis Coarsening of facial features Prognathism Macroglossia Arthritis Increased incidence of [source] Clinical findings Coarsened, enlarged facies, lips, tongue, nose, jaw, hands, feet, supraorbital ridge and frontal bones, widely spaced teeth, bone proliferation in extremities, soft tissue thickening, hyperhidrosis, macroglossia, headache, amenorrhoea [source] carcinoids, islet cell tumors, adrenal adenomas or other 'endocrine' tumors Clinical Coarsened, enlarged facies, lips, nose, jaw, hands, feet, and frontal bones, widely spaced teeth, bone proliferation in extremities, soft tissue thickening, hyperhidrosis, macroglossia [source]
  • Prognathism Symptoms due to excess of GH/IGF-I These include the following: Soft tissue swelling and enlargement of extremities Increase in ring and/or shoe size Hyperhidrosis Coarsening of facial features Prognathism Macroglossia Arthritis Increased incidence of [source] Prognathic mandible. [source] enlargement of the hands, feet, nose, tongue, lips and ears general thickening of the skin internal organs (especially heart and kidneys) vocal cords, resulting in a characteristic thick, deep voice and slowing of speech skull, frontal bossing mandible: prognathism [source]
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Entire body system
  • Coarse Facial Features Children with gigantism have few soft tissue effects (eg, peripheral edema, coarse facial features), because of their rapid linear growth. [source] A person with acromegaly usually has large hands and feet, thick lips, coarse facial features, a jutting forehead and jaw, and widely spaced teeth. [source] His growth-hormone excess manifested as tall stature, coarse facial features, and macrocephaly. [source]
  • Congestive Heart Failure heart failure, which may be due to uncontrolled hypertension or to an intrinsic form of cardiomyopathy attributable to excess GH/IGF-I Increased incidence of colonic polyps and adenocarcinoma of the colon In comparison with acromegalic patients with [source] Increased incidence of obstructive sleep apnea Increased incidence of glucose intolerance or frank diabetes mellitus, hypertension, and cardiovascular disease Hyperphosphatemia, hypercalcuria, and hypertriglyceridemia possible Increased incidence of congestive [source] The longterm prognosis is relatively poor, however, and most cats die of congestive heart failure, chronic renal failure, or signs of an expanding pituitary mass. [source]
  • Soft Tissue Swelling Symptoms due to excess of GH/IGF-I These include the following: Soft tissue swelling and enlargement of extremities Increase in ring and/or shoe size Hyperhidrosis Coarsening of facial features Prognathism Macroglossia Arthritis Increased incidence of [source] Soft tissue swelling in the back of the throat can cause snoring and sleep apnea, leading to disturbed sleep and daytime sleepiness. [source] Acromegaly also may cause soft tissue swelling which, when present in the hands, can cause carpal tunnel syndrome, pinching of the median nerve at the wrist, or neuropathy (nerve irritation). [source]
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Face, Head & Neck
  • Frontal Bossing Markers Typically shows elevated levels of: growth hormone IGF-1 (insulin growth factor 1) Plain radiograph Skull Calvarial thickening, frontal bossing, enlarged sinuses (especially frontal sinuses) and an enlarged sella turcica . [source] bossing mandible: prognathism with gaping teeth skin changes hypertrichosis hyperpigmentation hyperhidrosis Over 90% of cases are the result of a pituitary adenoma , usually a macroadenoma .  [source] physical signs: overgrowth enlargement of the hands, feet, nose, tongue, lips and ears general thickening of the skin internal organs (especially heart and kidneys) vocal cords, resulting in a characteristic thick, deep voice and slowing of speech skull, frontal [source]
  • Nose Enlargement Due to excess of GH: Gradual change in appearance due to the effects on cartilage and soft tissues: enlargement of hands and feet (increase in ring and shoe size), frontal bossing, thickening of the nose, enlarged tongue (macroglossia), growth of the [source]
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neurologic
  • Bitemporal Hemianopia Bitemporal hemianopia may develop if suprasellar extension compresses the optic chiasm. [source] Hypertension Diabetes mellitus (excess of GH leads to insulin resistance) Heart failure Kidney failure Colorectal cancer Compression of the optic chiasm leading to loss of vision in the outer visual fields (typically bitemporal hemianopia.) [source] Visual field defects: the most common defect is a bitemporal hemianopia[source]
  • Headache Headaches and visual field defects are the most common symptoms. [source] Features of gigantism include the following: Longitudinal acceleration of linear growth secondary to insulinlike growth factor I (IGF-I) excess is the cardinal clinical feature Tumor mass may cause headaches, visual changes due to optic nerve compression [source] Over time other symptoms such as headache and bony changes may decrease. [source]
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Skin
  • Coarseness of the Skin Symptoms may include: swelling of the hands and feet facial features become coarse as bones grow body hair becomes coarse as the skin thickens and/or darkens increased perspiration accompanied with body odor protruding jaw voice deepening enlarged lip [source] Acromegaly may produce the following signs and symptoms, which can vary from one person to another: Enlarged hands and feet Coarsened, enlarged facial features Coarse, oily, thickened skin Excessive sweating and body odor Small outgrowths of skin tissue [source] Typical symptoms include: joint pain large hands and feet carpal tunnel syndrome (compression of the nerve in the wrist, causing numbness and weakness of the hands) thick, coarse, oily skin skin tags enlarged lips, tongue and nose a protruding jaw and [source]
  • Hirsutism skin (sebaceous glands) causing oily skin, thickening of the skin, skin tags (growths) Sleep apnea Widened fingers or toes, with swelling, redness, and pain Other symptoms that may occur with this disease: Colon polyps Excess hair growth in females ( hirsutism [source] Large glands in the skin (sebaceous glands), thickening of the skin, skin tags (growths) Sleep apnea Widened fingers or toes, with swelling, redness, and pain Other symptoms that may occur with this disease: Colon polyps Excess hair growth in females ( hirsutism [source] (See "Patient education: Hirsutism (excess hair growth in women) (Beyond the Basics)" .) [source]
  • Hyperpigmentation general thickening of the skin internal organs (especially heart and kidneys) vocal cords, resulting in a characteristic thick, deep voice and slowing of speech skull, frontal bossing mandible: prognathism with gaping teeth skin changes hypertrichosis hyperpigmentation [source] face Noticeably large pores, acne-like comedones (blackheads) and thick swollen eyelids Increased body hair ( hypertrichosis ), excessive sweating ( hyperhidrosis ), and oily skin ( seborrhoea ) Increased number of skin tags and darkening of the skin ( hyperpigmentation [source] expansion of the skull at the fontanelle Pronounced brow protrusion, often with ocular distension (frontal bossing) Pronounced lower jaw protrusion (prognathism) with attendant macroglossia (enlargement of the tongue) and teeth spacing Hypertrichosis, hyperpigmentation [source]
  • Hypertrichosis lips and ears general thickening of the skin internal organs (especially heart and kidneys) vocal cords, resulting in a characteristic thick, deep voice and slowing of speech skull, frontal bossing mandible: prognathism with gaping teeth skin changes hypertrichosis [source] Enlarged swollen feet and hands The skin is thickened and may feel doughy Thick and hard nails Deepening of forehead creases and skin folds on the face Noticeably large pores, acne-like comedones (blackheads) and thick swollen eyelids Increased body hair ( hypertrichosis [source] speech Generalized expansion of the skull at the fontanelle Pronounced brow protrusion, often with ocular distension (frontal bossing) Pronounced lower jaw protrusion (prognathism) with attendant macroglossia (enlargement of the tongue) and teeth spacing Hypertrichosis [source]
  • Increased Sweating People with acromegaly may suffer with headaches, aches and pains in their bones and joints, and increased sweating[source]
  • Skin Thickening Hands and feet become enlarged; facial features are exaggerated as the jaw lengthens and the nose and forehead grow thicker; the skin thickens; and most internal organs enlarge. [source] Symptoms may include: swelling of the hands and feet facial features become coarse as bones grow body hair becomes coarse as the skin thickens and/or darkens increased perspiration accompanied with body odor protruding jaw voice deepening enlarged lip [source] , thickening of the skin, skin tags (growths) Sleep apnea Widened fingers or toes, with swelling, redness, and pain Other symptoms that may occur with this disease: Colon polyps Excess hair growth in females ( hirsutism ) High blood pressure Type 2 diabetes [source]
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respiratoric
  • Hoarseness the stool Carpal tunnel syndrome Decreased muscle strength ( weakness ) Decreased peripheral vision Easy fatigue Excessive height (when excess GH production begins in childhood) Excessive sweating Headache Heart enlargement, which can cause fainting Hoarseness [source] They include the following: Broadening of the nose, lips, ears, and forehead; enlarged tongue and increased spacing between front teeth Headaches Hoarse voice; sleep apnea and loud snoring Growth in hands and feet that requires larger shoes, rings, and [source] may include any of the following: Body odor Carpal tunnel syndrome Decreased muscle strength ( weakness ) Decreased peripheral vision Easy fatigue Excessive height (when excess growth hormone production begins in childhood) Excessive sweating Headache Hoarseness [source]
  • Sleep Apnea offensive body odor due to enlargement of the sweat glands Coarsening of facial features Fatigue and weakness in legs and arms Sleep apnea Arthritis and other joint problems particularly of the hips, knees and temporomandibular joint of the jaw Enlargement [source] apnea) Sinuses, vocal cords and soft tissues of the throat larynx (causing deep sonorous voice) Carpal tunnel syndrome, which causes numbness, tingling and pain in the hand and arm Skin change: Thickened, oily skin Severe acne Excessive sweating and [source] complications in adults may include the following: Abnormally large growth and deformity of the: Hands Feet Face (protrusion of brow and lower jaw) Jaw (overbite such that lower teeth close in front of upper teeth) Lips Tongue (causing snoring and obstructive sleep [source]
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musculoskeletal
  • Arthralgia Common side effects of Somatuline Depot include diarrhea, cholelithiasis, abdominal pain, nausea, injection site reactions, flatulence, arthralgia, and loose stools. [source] The growth related effects can also now be divided into three further categories for even further simplicifcation: Hard Tissue Growth Arthralgia due to joint tissue overgrowth Enlarged jaw ( macrognathia ) Frontal Bossing Widened space between lower incisor [source] Articular overgrowth of synovial tissue and arthropathy leading to arthralgia and osteoarthritis in 24%, back pain and kyphosis. [source]
  • Large Feet Acromegaly characteristically leads to swollen hands and large feet[source] They include: a large jaw and tongue gaps between the teeth a more prominent brow swollen hands large feet rough and oily skin skin tags Other changes include: tingling and lack of sensation in the hands and feet heavy sweating headaches a deeper voice [source] feet (change in shoe size), large hands (change in ring or glove size) Large glands in the skin (sebaceous glands) causing oily skin, thickening of the skin, skin tags (growths) Sleep apnea Widened fingers or toes, with swelling, redness, and pain Other [source]
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cardiovascular
Eyes
Ears

Workup

Measurement of:

  • GH
  • GHRH
  • TRH
  • Oral glucose
  • IGF-1
  • Prolactin

Imaging studies include MRI, CT scanning and radiography. Radiography reveals increase in length and thickness of certain bones like mandible and ribs. Mainly membranous bones are increased in size in radiographs of acromegalic patients. Increased cartilage growth is also evident. Histological findings are also important diagnostic tool and reveal different types of tumors in acromegaly.

Test Results

Visual Field Test

Laboratory

Serum
  • Hyperprolactinemia Tumor damage to the pituitary stalk may cause hyperprolactinemia due to loss of inhibitory regulation of prolactin secretion by the hypothalamus. [source] include the following: Longitudinal acceleration of linear growth secondary to insulinlike growth factor I (IGF-I) excess is the cardinal clinical feature Tumor mass may cause headaches, visual changes due to optic nerve compression, and hypopituitarism Hyperprolactinemia [source] hypertension or to an intrinsic form of cardiomyopathy attributable to excess GH/IGF-I Increased incidence of colonic polyps and adenocarcinoma of the colon In comparison with acromegalic patients with GH–secreting adenomas alone, patients who have hyperprolactinemia [source]
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Urine

Imaging

CT
MR

Treatment

Acromegaly can be treated surgically as well as by medications. Surgical removal of adenomas is quite successful nowadays [11] [12]. If surgery is not successful regarding GH hypersecretion then radiation therapy is used. Medications include mostly analogues of somatostatin like octreotide, which decrease the secretion of growth hormones and IGF-1, because somatostatin has an inhibitory effect on GH [13]. Dopamine agonists like bromocriptine are also employed as a pharmacological treatment because they bind to dopamine type 2 receptors in the pituitary gland and cause depressed secretion of GH [14].

Transsphenoidal surgery is used to completely remove the tumor. It is an efficient procedure and uses nasal approach to remove the tumor. After treatment, patients should be monitored throughout their life for their GH and IGF-1 levels. Also, patients should be evaluated for severe GH deficiency after surgery [15].

Prognosis

Mortality and morbidity rates are increased in acromegaly. Increased secretion of GH and IGF-1 has somatic and metabolic effects affecting all organ systems and patients may develop sleep apnea, hypertension, cardiomyopathy, nerve root compression, carpal tunnel syndrome, diabetes mellitus, and various other cerebrovascular, cardiovascular, and respiratory disorders [10]. In addition, the pituitary lesion may cause symptoms including headache and visual field defects. Early diagnosis and management of complications improves the long-term outcome [10].

Complications

  • Arthritis Symptoms due to excess of GH/IGF-I These include the following: Soft tissue swelling and enlargement of extremities Increase in ring and/or shoe size Hyperhidrosis Coarsening of facial features Prognathism Macroglossia Arthritis Increased incidence of [source] People with acromegaly can also suffer from high blood pressure, diabetes and arthritis[source] Other problems that affect the quality of life of people with acromegaly include arthritis, which can be progressive, and the fact that there is a possible increased risk of heart disease and sometimes bowel tumours. [source]
  • Carpal Tunnel Syndrome To understand better why patients with acromegaly develop the carpal tunnel syndrome[source] Adults with acromegaly are known to be at risk for a condition known as the carpal tunnel syndrome[source] It is not known whether acromegaly leads to the carpal tunnel syndrome because the soft tissues in the carpal tunnel swell and compress the median nerve (which seems to be the case in patients without acromegaly who develop the carpal tunnel syndrome[source]
  • Colonic Polyp Clin Endocrinol (Oxf) . 32 : 65 – 71 . 41 , Melmed S. 1991 Colon polyps in acromegaly. [source] Colon polyps in acromegaly Over the years, numerous retrospective and a few prospective studies have suggested an increased incidence of benign and precancerous colon polyps in patients with acromegaly ( 38 – 51 ) ( Table 3 ). [source] Clin Endocrinol (Oxf) . 47 : 23 – 28 . 47 , et al. 1997 Acromegaly, colonic polyps and carcinoma. [source]
  • Cranial Nerve Palsy References: [1] [4] Clinical features Tumor mass effects Headache , vision loss ( bitemporal hemianopsia ) , cranial nerve palsies : Oligomenorrhea , secondary amenorrhea , galactorrhea , vaginal atrophy : Erectile dysfunction , decreased libido, testicular [source] These include headaches, visual abnormalities, typically bitemporal hemianopsia, and cranial nerve palsies[source] . • After 10 years, about half of all patients receiving radiotherapy have signs of pituitary trophic hormone disruption • hair loss, cranial nerve palsies, tumor necrosis with hemorrhage, and, rarely, loss of vision or pituitary apoplexy, have been documented [source]
  • Diabetes Mellitus Net weight gain of lean body mass in cats with uncontrolled diabetes mellitus is a key sign of acromegaly. [source] In a recent study, acromegaly was the most common (25%) underlying cause of non-insulin–dependent diabetes mellitus in cats, and it should be suspected in any cat that does not respond to standard therapy for type 2 diabetes mellitus (high-protein, low-carbohydrate [source] Impaired glucose tolerance and insulin resistance resulting in diabetes mellitus are seen in all cats with acromegaly. [source]
  • Gigantism Gigantism The presentation of patients with gigantism is usually dramatic, unlike the insidious onset of acromegaly in adults. [source] Children with gigantism have few soft tissue effects (eg, peripheral edema, coarse facial features), because of their rapid linear growth. [source] Features of gigantism include the following: Longitudinal acceleration of linear growth secondary to insulinlike growth factor I (IGF-I) excess is the cardinal clinical feature Tumor mass may cause headaches, visual changes due to optic nerve compression [source]
  • Hypercalciuria Metabolic features • Impaired glucose tolerance • Diabetes mellitus • Insulin resistance • Other endocrine consequences • Goiter • Hypercalciuria • Galactorrhea • Decrease libido, impotence • Menstrual abnormalities Bone and joint manifestations • Increased [source] and nephrolithiasis may occur in 6–77% of patients with acromegaly. • Proposed mechanisms of hypercalciuria include parathyroid hyperplasia, renal tubular acidosis, increased calcium absorption, and overproduction of 1, 25 (OH)2 D 43. • One study evaluated [source] as a result of the stimulating effects IGF-I on thyrocyte growth. 42. • Hypercalcemia in acromegaly is reported in up to 8% of patients; • it is usually secondary to coexistent hyperparathyroidism and does not resolve after treatment of excess GH. • hypercalciuria [source]
  • Hyperglycemia Excess GH: 1) stimulates gluconeogenesis and lipolysis, causing hyperglycemia and elevated free fatty acid levels; 2) leads to both hepatic and peripheral insulin resistance, with compensatory hyperinsulinemia. [source] The catabolic actions of growth hormone include insulin antagonism and lipolysis, with the net effect of promoting hyperglycemia[source] Despite severe insulin resistance and hyperglycemia, ketosis is rare. [source]
  • Hyperphosphatemia or shoe size Hyperhidrosis Coarsening of facial features Prognathism Macroglossia Arthritis Increased incidence of obstructive sleep apnea Increased incidence of glucose intolerance or frank diabetes mellitus, hypertension, and cardiovascular disease Hyperphosphatemia [source] Hyperphosphatemia without azotemia is also a common clinicopathologic finding. [source] GH increases tubular reabsorption of phosphate and leads to mild hyperphosphatemia[source]
  • Hypertension , and cardiovascular disease Hyperphosphatemia, hypercalcuria, and hypertriglyceridemia possible Increased incidence of congestive heart failure, which may be due to uncontrolled hypertension or to an intrinsic form of cardiomyopathy attributable to excess [source] enlargement of extremities Increase in ring and/or shoe size Hyperhidrosis Coarsening of facial features Prognathism Macroglossia Arthritis Increased incidence of obstructive sleep apnea Increased incidence of glucose intolerance or frank diabetes mellitus, hypertension [source] They may also experience headaches and can often suffer from diabetes, hypertension, and heart failure. [source]
  • Hypertriglyceridemia of facial features Prognathism Macroglossia Arthritis Increased incidence of obstructive sleep apnea Increased incidence of glucose intolerance or frank diabetes mellitus, hypertension, and cardiovascular disease Hyperphosphatemia, hypercalcuria, and hypertriglyceridemia [source]
  • Impaired Glucose Tolerance Impaired glucose tolerance and insulin resistance resulting in diabetes mellitus are seen in all cats with acromegaly. [source] Overt diabetes mellitus (DM) has been reported in 13-30% of acromegalic patients [6] , while impaired glucose tolerance was noted amongst 36% acromegalic patients in another series [7] . [source] Impaired glucose tolerance occurs in nearly half the patients with acromegaly and in gigantism, but clinically significant diabetes mellitus occurs in only about 10% of patients. [source]
  • Kyphosis Also, bones and viscera increase in size including nose, cranium, supraorbital ridges, forehead and vertebrae, causing symptoms such as macrognathia, kyphosis, goiter and left ventricular hypertrophy. [source] Articular overgrowth of synovial tissue and arthropathy leading to arthralgia and osteoarthritis in 24%, back pain and kyphosis[source] Trunk Bony deformation also affects the spine, with upper dorsal kyphosis and compensatory lumbar hyperlordosis. [source]
  • Obstructive Sleep Apnea sleep apnea Increased incidence of glucose intolerance or frank diabetes mellitus, hypertension, and cardiovascular disease Hyperphosphatemia, hypercalcuria, and hypertriglyceridemia possible Increased incidence of congestive heart failure, which may [source] Symptoms due to excess of GH/IGF-I These include the following: Soft tissue swelling and enlargement of extremities Increase in ring and/or shoe size Hyperhidrosis Coarsening of facial features Prognathism Macroglossia Arthritis Increased incidence of obstructive [source] sleep apnea) Sinuses, vocal cords and soft tissues of the throat larynx (causing deep sonorous voice) Carpal tunnel syndrome, which causes numbness, tingling and pain in the hand and arm Skin change: Thickened, oily skin Severe acne Excessive sweating [source]
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Etiology

Acromegaly is caused by:

  • Increased secretion of growth hormone (GH) caused by pituitary adenomas in most cases.
  • Hypothalamic tumors which result in increased secretion of growth hormone releasing hormone (GHRH).
  • IGF-1 (insulin-like growth factor 1) oversecretion, which is a growth regulating substance produced by the liver under the effect of growth hormone [2].
  • In some instances, tumors of the adrenal glands or lungs may cause ectopic secretion of GH, rarely of GHRH.

Epidemiology

The prevalence of acromegaly in Europe has been reported to be 30-70:1,000,000 [3] [4], however, recent studies observed higher numbers [4] [5] [6]. Acromegaly affects both males and females equally. The mean age for diagnosis is 40 years in males and 45 years in females.

Sex distribution
Age distribution

Pathophysiology

Growth hormone, which is released by the adenohypophysis, is regulated by the hypothalamus through growth hormone releasing hormone and somatostatin. GH exhibits its regulatory effects by insulin-like growth factor I, which is produced by the liver under the influence of GH. Most cases of acromegaly are caused by pituitary adenomas, resulting in increased secretion of GH and insulin-like growth factor I subsequently. Both cause increased growth of soft tissues, bones and viscera. Because somatostatin decreases the secretion of GH, drugs mimicking the actions of somatostatin are given to acromegalic patients to decrease GH.

Extrapituitary tumours including neuroendocrine tumors are rare causes and may also increase the secretion of GH in the same way and causing the same results [7] [8] [9]. Tumors associated with GHRH production such intracranial gangliocytomas and disruption of the somatostatin feedback pathway also lead to acromegaly.

Prevention

Acromegaly can not be prevented. Early diagnosis should is important to avoid severe complications.

Summary

Acromegaly is most often caused by adenomas of the pituitary gland. If the tumour occurs before puberty, linear growth increases, a condition known as pituitary gigantism. After adolescence, when the fusion of the epiphyseal growth plates has occured, acromegaly develops. The disorder causes acral and soft tissue overgrowth, characterized by markedly increased growth of hands and feet. Also, bones and viscera increase in size including nose, cranium, supraorbital ridges, forehead and vertebrae, causing symptoms such as macrognathia, kyphosis, goiter and left ventricular hypertrophy [1].

Patient Information

Definition

Acromegaly is usually caused by a tumor of the pituitary gland which results in increased growth hormone secretion. This leads to secondary effects due to growth hormone overproduction.

Cause

The common cause of acromegaly is a pituitary adenoma that causes increased growth hormone (GH) and subsequent insulin-like growth factor 1 (IGF-1) secretion.

Signs and symptoms

The symptoms by which the acromegaly patient can be recognized are increased bone growth, hunched back, increased blood pressure, protrusion of jaw and forehead and increased growth of extremities.

Diagnosis

Diagnosis is made by measuring levels of GH and IGF-1 in the blood. Also radiographic and other imaging techniques are used.

Treatment

Acromegaly can be treated medically as well as surgically. Medical treatment is by use of somatostatin analogues, dopamine agonists and growth hormone antagonists. Radiation is also used.

Self-assessment

References

  1. Giustina A, Chanson P, Bronstein MD, Klibanski A, Lamberts S, Casanueva FF, et al. A consensus on criteria for cure of acromegaly. J Clin Endocrinol Metab. Jul 2010;95(7):3141-8.
  2. Soares BS, Eguchi K, Frohman LA. Tumor deletion mapping on chromosome 11q13 in eight families with isolated familial somatotropinoma and in 15 sporadic somatotropinomas. J Clin Endocrinol Metab. Dec 2005;90(12):6580-7.
  3. Bengtsson BA, Edén S, Ernest I, et al. Epidemiology and long-term survival in acromegaly. A study of 166 cases diagnosed between 1955 and 1984. Acta Med Scand 1988; 223:327.
  4. Ribeiro-Oliveira A Jr, Barkan A. The changing face of acromegaly--advances in diagnosis and treatment. Nat Rev Endocrinol 2012; 8:605.
  5. Fernandez A, Karavitaki N, Wass JA. Prevalence of pituitary adenomas: a community-based, cross-sectional study in Banbury (Oxfordshire, UK). Clin Endocrinol (Oxf) 2010; 72:377.
  6. Daly AF, Rixhon M, Adam C, et al. High prevalence of pituitary adenomas: a cross-sectional study in the province of Liege, Belgium. J Clin Endocrinol Metab 2006; 91:4769.
  7. Melmed S, Ezrin C, Kovacs K, et al. Acromegaly due to secretion of growth hormone by an ectopic pancreatic islet-cell tumor. N Engl J Med 1985; 312:9.
  8. Beuschlein F, Strasburger CJ, Siegerstetter V, et al. Acromegaly caused by secretion of growth hormone by a non-Hodgkin's lymphoma. N Engl J Med 2000; 342:1871.
  9. Altstadt TJ, Azzarelli B, Bevering C, et al. Acromegaly caused by a growth hormone-releasing hormone-secreting carcinoid tumor: case report. Neurosurgery 2002; 50:1356.
  10. Melmed S, Casanueva FF, Klibanski A, Bronstein MD, Chanson P, Lamberts SW, et al. A consensus on the diagnosis and treatment of acromegaly complications. Pituitary. 2013 Sep. 16(3):294-302.
  11. Ross DA, Wilson CB. Results of transsphenoidal microsurgery for growth hormone-secreting pituitary adenoma in a series of 214 patients. J Neurosurg 1988; 68:854.
  12. Fahlbusch R, Honegger J, Buchfelder M. Surgical management of acromegaly. Endocrinol Metab Clin North Am 1992; 21:669.
  13. Shimon I, Yan X, Taylor JE, et al. Somatostatin receptor (SSTR) subtype-selective analogues differentially suppress in vitro growth hormone and prolactin in human pituitary adenomas. Novel potential therapy for functional pituitary tumors. J Clin Invest 1997; 100:2386.
  14. Sandret L, Maison P, Chanson P. Place of cabergoline in acromegaly: a meta-analysis. J Clin Endocrinol Metab. 2011 May. 96(5):1327-35.
  15. Ronchi CL, Giavoli C, Ferrante E, et al. Prevalence of GH deficiency in cured acromegalic patients: impact of different previous treatments. Eur J Endocrinol 2009; 161:37.



Media References

  1. Acromegaly facial features, CC BY 2.0

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