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.

Skin
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.[britannica.com]
  • 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[hopkinsmedicine.org]
  • , 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[medlineplus.gov]
  • Definition (NCI) A syndrome characterized by enlargement of the hands, feet, ears, nose, lips, and tongue, skin thickening and swelling of internal organs.[fpnotebook.com]
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[medlineplus.gov]
  • (See "Patient education: Hirsutism (excess hair growth in women) (Beyond the Basics)" .)[uptodate.com]
  • Women may have mild hirsutism.[patient.info]
  • In young women, acromegaly and the polycystic ovarian syndrome may need to be differentiated as they share some common features such as hirsutism and menstrual irregularity.[clinicaladvisor.com]
Coarseness of the Skin
  • 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[mayoclinic.org]
  • 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[hopkinsmedicine.org]
  • As time goes on, common symptoms include: abnormally large hands and feet large, prominent facial features (such as the nose and lips) and an enlarged tongue skin changes – such as thick, coarse, oily skin; skin tags ; or sweating too much deepening of[nhs.uk]
  • Other symptoms of acromegaly include thick, coarse, oily skin; skin tags; enlarged lips, nose and tongue; deepening of the voice due to enlarged sinuses and vocal cords; snoring due to upper airway obstruction; excessive sweating and skin odor; fatigue[pituitary.mgh.harvard.edu]
Hyperpigmentation
  • […] including cutis verticis gyrata ) 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[radiopaedia.org]
  • […] 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[dermnetnz.org]
  • […] 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[en.wikipedia.org]
  • […] takes on a triangular configuration) Wide spacing of the teeth and prognathism Cutis verticis gyrata (ie, furrows resembling gyri of the scalp) [6] Small sessile and pedunculated fibromas (ie, skin tags) Hypertrichosis Oily skin (acne is not common) Hyperpigmentation[emedicine.medscape.com]
Hypertrichosis
  • […] the skin (including cutis verticis gyrata ) 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[radiopaedia.org]
  • 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[dermnetnz.org]
  • […] 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[en.wikipedia.org]
  • Enlargement of the lower lip and nose (the nose takes on a triangular configuration) Wide spacing of the teeth and prognathism Cutis verticis gyrata (ie, furrows resembling gyri of the scalp) [6] Small sessile and pedunculated fibromas (ie, skin tags) Hypertrichosis[emedicine.medscape.com]
Increased Sweating
  • People with acromegaly may suffer with headaches, aches and pains in their bones and joints, and increased sweating.[yourhormones.info]
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  • Ears
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  • Eyes
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  • cardiovascular
    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[emedicine.medscape.com]
    • They may also experience headaches and can often suffer from diabetes, hypertension, and heart failure.[pfizer.com]
    • Complications from increased growth hormone levels include atherosclerosis, peripheral neuropathy, hypertension, hyperglycemia, airway obstruction, cardiomyopathy, and visceromegaly involving the salivary glands, liver, spleen, and kidneys.[medical-dictionary.thefreedictionary.com]
    • References: [1] [7] [8] Complications Complications lead to increased mortality Cardiovascular complications ( CHF , hypertension , arrhythmia, valvular disease, hypertrophy): the main cause of death Diabetes mellitus Sleep apnea Neoplastic disease (e.g[amboss.com]
    • Complications Hypertension, coronary heart disease, cardiac failure, cerebrovascular disease.[patient.info]
    Chest Pain
    • Symptoms of slow heart rate may include dizziness or lightheadedness, fainting or near-fainting, chest pain, shortness of breath, confusion or memory problems, and weakness or extreme tiredness.[acromegaly.somatulinedepot.com]
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  • musculoskeletal
    Arthritis
    • If not treated, acromegaly can lead to heart problems, arthritis, diabetes, high blood pressure, or even death.[cedars-sinai.edu]
    • 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[emedicine.medscape.com]
    • People with acromegaly can also suffer from high blood pressure, diabetes and arthritis.[yourhormones.info]
    • […] with acromegaly E22.0 in (due to) acromegaly E22.0 - see also subcategory M14.8- Arthropathy M12.9 - see also Arthritis ICD-10-CM Diagnosis Code M12.9 Arthropathy, unspecified 2016 2017 2018 Billable/Specific Code in (due to) acromegaly E22.0 Erdheim's[icd10data.com]
    • […] 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, nose, and tongue thickened ribs (creating a barrel chest) joint pain degenerative arthritis[hopkinsmedicine.org]
    Arthralgia
    • Must be screened for in the presence of acral growth, coarsening of facial features, and when suspected in conjunction with commonly associated conditions, such as carpal tunnel syndrome, arthralgia, glucose intolerance or diabetes, amenorrhoea, HTN,[bestpractice.bmj.com]
    • Common side effects of Somatuline Depot include diarrhea, cholelithiasis, abdominal pain, nausea, injection site reactions, flatulence, arthralgia, and loose stools.[acromegaly.org]
    • Articular overgrowth of synovial tissue and arthropathy leading to arthralgia and osteoarthritis in 24%, back pain and kyphosis.[patient.info]
    • 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[theartofmed.wordpress.com]
    • […] tolerance • Diabetes mellitus • Insulin resistance • Other endocrine consequences • Goiter • Hypercalciuria • Galactorrhea • Decrease libido, impotence • Menstrual abnormalities Bone and joint manifestations • Increased articular cartilage thickness • Arthralgias[slideshare.net]
    Large Feet
    • Symptoms Acromegaly characteristically leads to swollen hands and large feet.[medicalnewstoday.com]
    • 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[medlineplus.gov]
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  • respiratoric
    Sleep Apnea
    • These are the changes that patients can notice, and some of these changes, like sleep apnea, can be life threatening.[urmc.rochester.edu]
    • […] 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[neurosciences.uvahealth.com]
    • 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 be due[emedicine.medscape.com]
    • It causes some degree of tumor shrinkage in 30-50% of patients, and often improves symptoms of soft tissue swelling, headache, joint pains and sleep apnea.[acromegaly.org]
    • References: [1] [7] [8] Complications Complications lead to increased mortality Cardiovascular complications ( CHF , hypertension , arrhythmia, valvular disease, hypertrophy): the main cause of death Diabetes mellitus Sleep apnea Neoplastic disease (e.g[amboss.com]
    Hoarseness
    • Ultimately they cause considerable disability (aside from the need for larger rings, gloves, and shoes) including hoarseness , sleep apnea , joint pain, cardiovascular disease, hypertension, insulin resistance , visual impairment and severe headaches.[medicinenet.com]
    • […] 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[medlineplus.gov]
    • 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[pfizer.com]
    • […] enlarged jaw or tongue a prominent brow excessive growth spurts, which are more common in people who’ve had abnormal growth before adolescence weight gain swollen and painful joints that limit movement spaces between the teeth splayed fingers and toes a hoarse[healthline.com]
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  • neurologic
    Headache
    • Headaches and visual field defects are the most common symptoms.[emedicine.medscape.com]
    • Short-term side-effects – such as blurred vision, headaches and lethargy – may also occur.[yourhormones.info]
    • Some of the symptoms of acromegaly, such as headaches and impaired vision, are due to the tumor mass pressing on nearby brain tissues.[mayoclinic.org]
    • They may also experience headaches and can often suffer from diabetes, hypertension, and heart failure.[pfizer.com]
    Bitemporal Hemianopsia
    • The most common manifestation is a bitemporal hemianopsia caused by pressure on the optic chiasm.[emedicine.medscape.com]
    • 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[amboss.com]
    • Visual studies suggest bitemporal hemianopsia.[theartofmed.wordpress.com]
    • Vision problems can include: A loss of the outer peripheral vision, called a bitemporal hemianopsia When severe, a patient can only see what is directly in front of them Many patients do not become aware of their visual loss until it is quite severe.[pituitary.ucla.edu]
    • Visual disorders When the adenoma grows upwards it can compress the optic chiasm, leading to visual field disturbances, which begin in the mid-periphery of the superior temporal sectors, then progress to bitemporal hemianopsia (Figure 6 ).[ojrd.biomedcentral.com]
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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 .[radiopaedia.org]
    • Untreated acromegaly results in marked bony and soft tissue changes including an altered facial appearance (frontal bossing, prognathism), enlargement of the hands and feet, sleep apnea, and carpal tunnel syndrome.[acromegaly.org]
    • Symptoms In acromegaly, elevated growth hormone causes an increase in shoe and ring size, enlargement of the mandible leading to an underbite, frontal bossing, and enlargement of the nose.[ccpd.ucsf.edu]
    • 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[patient.info]
    • 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[theartofmed.wordpress.com]
    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[patient.info]
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  • Entire body system
    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[emedicine.medscape.com]
    • Soft tissue swelling in the back of the throat can cause snoring and sleep apnea, leading to disturbed sleep and daytime sleepiness.[empoweryourhealth.org]
    • However, soft-tissue swelling occurs, and the peripheral nerves are enlarged.[merckmanuals.com]
    • tissue swelling visibly resulting in enlargement of the hands, feet, nose, lips and ears, and a general thickening of the skin Soft tissue swelling of internal organs, notably the heart with attendant weakening of its muscularity, and the kidneys, also[en.wikipedia.org]
    • When surgery is successful, soft tissue swelling may decrease in just a few days.[rarediseases.org]
    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[emedicine.medscape.com]
    • 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.[msdvetmanual.com]
    • Acromegalic subjects may develop congestive heart failure , particularly when blood pressure becomes high.[britannica.com]
    • Cardiovascular signs include the presence of a heart murmur, hypertension, arrhythmia, and congestive heart failure associated with hypertrophic cardiomyopathy. 3 Neurologic disease associated with feline acromegaly is uncommon but can occur with large[veterinarymedicine.dvm360.com]
    • heart failure Pulmonary: [1] [2] [5] [6] Obstructive sleep apnea Macroglossia Upper airway obstruction Ventilatory dysfunction Upper airway obstruction Metabolic: [1] [2] [6] Insulin resistance Impaired glucose metabolism Diabetes mellitus Visceral:[physio-pedia.com]
    Coarse Facial Features
    • Children with gigantism have few soft tissue effects (eg, peripheral edema, coarse facial features), because of their rapid linear growth.[emedicine.medscape.com]
    • A person with acromegaly usually has large hands and feet, thick lips, coarse facial features, a jutting forehead and jaw, and widely spaced teeth.[hormone.org]
    • His growth-hormone excess manifested as tall stature, coarse facial features, and macrocephaly.[emedicine.medscape.com]
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  • Jaw & Teeth
    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[emedicine.medscape.com]
    • Untreated acromegaly results in marked bony and soft tissue changes including an altered facial appearance (frontal bossing, prognathism), enlargement of the hands and feet, sleep apnea, and carpal tunnel syndrome.[acromegaly.org]
    • It is characterized by bony enlargement of the FACE; lower jaw (PROGNATHISM); hands; FEET; HEAD; and THORAX.[fpnotebook.com]
    • Gradual enlargement of paranasal sinuses, prominence of nose and supraorbital ridges, prognathism, widely separated teeth, and an underbite are part of the coarsening of facial features.[medical-dictionary.thefreedictionary.com]
    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[emedicine.medscape.com]
    • 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[medical-dictionary.thefreedictionary.com]
    • […] hemianopsia ) , cranial nerve palsies : Oligomenorrhea , secondary amenorrhea , galactorrhea , vaginal atrophy : Erectile dysfunction , decreased libido, testicular volume Soft tissue effects Doughy skin texture, hyperhidrosis Deepening of the voice, macroglossia[amboss.com]
    • Macroglossia may cause obstructive sleep apnoea leading to daytime tiredness.[patient.info]
    • Cardiovascular features • Hypertrophy (biventricular or asymmetric septal) • Congestive Heart Failure (systolic and/or diastolic) • Coronary disease • Arrhythmias • Hypertension • Cardiomyopathy Respiratory manifestations • Macroglossia • Jaw malocclusion[slideshare.net]
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  • 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.

    Laboratory

    Urine
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  • Serum
    Hyperprolactinemia
    • Tumor damage to the pituitary stalk may cause hyperprolactinemia due to loss of inhibitory regulation of prolactin secretion by the hypothalamus.[emedicine.medscape.com]
    • Thus, in a case where a patient had Acromegaly AND Hyperprolactinemia due to a Somatotrope Adenoma that produces both, the symptoms of hyperprolactinemia would usually appear first and be much more prominent.[theartofmed.wordpress.com]
    • . • These patients present with clinical features of acromegaly as well as hyperprolactinemia, Cushing’s disease, or, rarely hyperthyroxinemia. 12.[slideshare.net]
    • Galactorrhea occurs in some women with acromegaly, usually in association with hyperprolactinemia .[merckmanuals.com]
    • Any patient with hyperprolactinemia should have a serum IGF-I level measured.[clinicaladvisor.com]
    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[emedicine.medscape.com]
    • A 2009 study suggests that in patients with acromegaly, insulin resistance and hyperinsulinemia are positively correlated with the level of disease activity. [ 17 ] Hypertriglyceridemia is found in 19-44% of patients.[emedicine.medscape.com]
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  • Imaging

    X-ray
    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[emedicine.medscape.com]
    • 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.[msdvetmanual.com]
    • Acromegalic subjects may develop congestive heart failure , particularly when blood pressure becomes high.[britannica.com]
    • Cardiovascular signs include the presence of a heart murmur, hypertension, arrhythmia, and congestive heart failure associated with hypertrophic cardiomyopathy. 3 Neurologic disease associated with feline acromegaly is uncommon but can occur with large[veterinarymedicine.dvm360.com]
    • heart failure Pulmonary: [1] [2] [5] [6] Obstructive sleep apnea Macroglossia Upper airway obstruction Ventilatory dysfunction Upper airway obstruction Metabolic: [1] [2] [6] Insulin resistance Impaired glucose metabolism Diabetes mellitus Visceral:[physio-pedia.com]
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  • MR
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  • CT
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  • Test Results

    Visual Field Test
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  • 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
    • If not treated, acromegaly can lead to heart problems, arthritis, diabetes, high blood pressure, or even death.[cedars-sinai.edu]
    • 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[emedicine.medscape.com]
    • People with acromegaly can also suffer from high blood pressure, diabetes and arthritis.[yourhormones.info]
    • […] with acromegaly E22.0 in (due to) acromegaly E22.0 - see also subcategory M14.8- Arthropathy M12.9 - see also Arthritis ICD-10-CM Diagnosis Code M12.9 Arthropathy, unspecified 2016 2017 2018 Billable/Specific Code in (due to) acromegaly E22.0 Erdheim's[icd10data.com]
    • […] 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, nose, and tongue thickened ribs (creating a barrel chest) joint pain degenerative arthritis[hopkinsmedicine.org]
    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[emedicine.medscape.com]
    • 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.[msdvetmanual.com]
    • Acromegalic subjects may develop congestive heart failure , particularly when blood pressure becomes high.[britannica.com]
    • Cardiovascular signs include the presence of a heart murmur, hypertension, arrhythmia, and congestive heart failure associated with hypertrophic cardiomyopathy. 3 Neurologic disease associated with feline acromegaly is uncommon but can occur with large[veterinarymedicine.dvm360.com]
    • heart failure Pulmonary: [1] [2] [5] [6] Obstructive sleep apnea Macroglossia Upper airway obstruction Ventilatory dysfunction Upper airway obstruction Metabolic: [1] [2] [6] Insulin resistance Impaired glucose metabolism Diabetes mellitus Visceral:[physio-pedia.com]
    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[emedicine.medscape.com]
    • They may also experience headaches and can often suffer from diabetes, hypertension, and heart failure.[pfizer.com]
    • Complications from increased growth hormone levels include atherosclerosis, peripheral neuropathy, hypertension, hyperglycemia, airway obstruction, cardiomyopathy, and visceromegaly involving the salivary glands, liver, spleen, and kidneys.[medical-dictionary.thefreedictionary.com]
    • References: [1] [7] [8] Complications Complications lead to increased mortality Cardiovascular complications ( CHF , hypertension , arrhythmia, valvular disease, hypertrophy): the main cause of death Diabetes mellitus Sleep apnea Neoplastic disease (e.g[amboss.com]
    • Complications Hypertension, coronary heart disease, cardiac failure, cerebrovascular disease.[patient.info]
    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.[hopkinsguides.com]
    • The catabolic actions of growth hormone include insulin antagonism and lipolysis, with the net effect of promoting hyperglycemia.[veterinarymedicine.dvm360.com]
    • Despite severe insulin resistance and hyperglycemia, ketosis is rare.[msdvetmanual.com]
    • ., small cell lung carcinoma , medullary thyroid cancer ) References: [3] [2] Pathophysiology Physiology of GH and IGF-1 GH secretion induced by stress, sport, and hypoglycemia ; inhibited especially by hyperglycemia or food intake Hypothalamus secretes[amboss.com]
    • Complications from increased growth hormone levels include atherosclerosis, peripheral neuropathy, hypertension, hyperglycemia, airway obstruction, cardiomyopathy, and visceromegaly involving the salivary glands, liver, spleen, and kidneys.[medical-dictionary.thefreedictionary.com]
    Diabetes Mellitus
    • Net weight gain of lean body mass in cats with uncontrolled diabetes mellitus is a key sign of acromegaly.[msdvetmanual.com]
    • Diabetes mellitus appears in 20 to 40 percent of acromegalic subjects because excess growth hormone blocks the action of insulin .[britannica.com]
    • Overt diabetes mellitus develops in about 20%, and glucose intolerance in about 40%, of patients with acromegaly.[diapedia.org]
    • Later joint pain, weakness, and sometimes diabetes mellitus and visual disturbances are seen.[medical-dictionary.thefreedictionary.com]
    • In one study, 13 of 14 cats with acromegaly were males, with an average age of 10.2 years. 2 This association may be biased, however, as most cats in which acromegaly is diagnosed are presented for complications associated with diabetes mellitus, which[veterinarymedicine.dvm360.com]
    Gigantism
    • Gigantism The presentation of patients with gigantism is usually dramatic, unlike the insidious onset of acromegaly in adults.[emedicine.medscape.com]
    • Onset of gigantism is usually at puberty , although some cases of toddlers and young children with gigantism have been reported.[encyclopedia.com]
    • 2018 Billable/Specific Code Applicable To Constitutional gigantism E34.4 ) constitutional tall stature ( ICD-10-CM Diagnosis Code E34.4 Constitutional tall stature 2016 2017 2018 Billable/Specific Code Applicable To Constitutional gigantism E34.4 ) increased[icd10data.com]
    • Male predominance was seen among AIP -mutated gigantism, whereas X-LAG had a strong female predilection. [ 5 ] Signs and symptoms Gigantism The presentation of patients with gigantism is usually dramatic, unlike the insidious onset of acromegaly in adults[emedicine.medscape.com]
    • When there is secretion of too much growth hormone before the end of adolescence, gigantism results.[medicinenet.com]
    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[emedicine.medscape.com]
    • Hyperphosphatemia without azotemia is also a common clinicopathologic finding.[msdvetmanual.com]
    • GH increases tubular reabsorption of phosphate and leads to mild hyperphosphatemia.[merckmanuals.com]
    • Hyperphosphatemia Treatment of Acromegaly Now let’s think back about how we can treat acromegaly.[theartofmed.wordpress.com]
    • Acromegalic cardiomyopathy (with dysfunction and arrhythmias) Left ventricular hypertrophy Increased left ventricular mass Disorders of calcium and bone metabolism The following calcium and bone metabolism disorders can be found in acromegaly: Hypercalciuria Hyperphosphatemia[emedicine.medscape.com]
    Carpal Tunnel Syndrome
    • To understand better why patients with acromegaly develop the carpal tunnel syndrome.[annals.org]
    • Must be screened for in the presence of acral growth, coarsening of facial features, and when suspected in conjunction with commonly associated conditions, such as carpal tunnel syndrome, arthralgia, glucose intolerance or diabetes, amenorrhoea, HTN,[bestpractice.bmj.com]
    • Untreated acromegaly results in marked bone and soft tissue changes including altered facial appearance, enlargement of the hands and feet, sleep apnoea, and carpal tunnel syndrome.[amrytpharma.com]
    • Untreated acromegaly results in marked bony and soft tissue changes including an altered facial appearance (frontal bossing, prognathism), enlargement of the hands and feet, sleep apnea, and carpal tunnel syndrome.[acromegaly.org]
    • tunnel syndrome, sleep apnea.[medical-dictionary.thefreedictionary.com]
    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[emedicine.medscape.com]
    • 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[neurosciences.uvahealth.com]
    • sleep apnea Carpal tunnel syndrome Skeletal effects Large skull, coarsened features ; : enlarged nose, forehead, and jaw (macrognathia) with diastema Widened hands, fingers, and feet Painful arthropathy (ankles, knees, hips, spine) Cardiovascular disease[amboss.com]
    • […] acromegaly, obstructive sleep apnea predominates over central sleep apnea 41. • Hyperprolactinemia with or without galactorrhea develops in approximately 30% of patients due to of pituitary stalk compression or mixed tumor secretion of GH and PRL. •[slideshare.net]
    • sleep apnea Macroglossia Upper airway obstruction Ventilatory dysfunction Upper airway obstruction Metabolic: [1] [2] [6] Insulin resistance Impaired glucose metabolism Diabetes mellitus Visceral: [1] [6] Organ enlargement Colon polyps Fluid retention[physio-pedia.com]
    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[emedicine.medscape.com]
    • A 2009 study suggests that in patients with acromegaly, insulin resistance and hyperinsulinemia are positively correlated with the level of disease activity. [ 17 ] Hypertriglyceridemia is found in 19-44% of patients.[emedicine.medscape.com]
    Kyphosis
    • Articular overgrowth of synovial tissue and arthropathy leading to arthralgia and osteoarthritis in 24%, back pain and kyphosis.[patient.info]
    • Trunk Bony deformation also affects the spine, with upper dorsal kyphosis and compensatory lumbar hyperlordosis.[ojrd.biomedcentral.com]
    • Associated complications include the following [ 15 ] : Acromegalic heart Increased muscle and soft tissue mass Increased kidney size Articular overgrowth of synovial tissue and hypertrophic arthropathy Joint symptoms, back pain, and kyphosis: Common[emedicine.medscape.com]
    Colonic Polyp
    • Clin Endocrinol (Oxf) . 32 : 65 – 71 . 41 , Melmed S. 1991 Colon polyps in acromegaly.[doi.org]
    • polyps and adenocarcinoma of the colon In comparison with acromegalic patients with GH–secreting adenomas alone, patients who have hyperprolactinemia as well tend to have an earlier onset of disease, lesser acromegalic features, and lower GH levels,[emedicine.medscape.com]
    • Internally, acromegaly causes organs to enlarge and can contribute to the development of diabetes, heart disease, and even colon polyps.[weillcornellbrainandspine.org]
    • Patients with acromegaly may have accelerated cardiovascular disease and thickening (hypertrophy) of the heart muscles, diabetes, and an increased risk of colon polyps.[ccpd.ucsf.edu]
    • Other tissue in the body also can become enlarged, causing symptoms such as: Carpal tunnel syndrome Deepening of the voice Diabetes Excessive sweating and changes in body odor Increased risk of colon polyps Joint pain and stiffness Sleep apnea and snoring[ucsfhealth.org]
    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[amboss.com]
    • . • 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[slideshare.net]
    Impaired Glucose Tolerance
    • These may include: Heart disease Arthritis Diabetes and impaired glucose tolerance High blood pressure The disease also raises your risk for colon polyps.[cedars-sinai.edu]
    • Impaired glucose tolerance and insulin resistance resulting in diabetes mellitus are seen in all cats with acromegaly.[msdvetmanual.com]
    • 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] .[diapedia.org]
    • 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.[merckmanuals.com]
    • 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[slideshare.net]
    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[slideshare.net]
    • Hypertension Acromegalic cardiomyopathy (with dysfunction and arrhythmias) Left ventricular hypertrophy Increased left ventricular mass Disorders of calcium and bone metabolism The following calcium and bone metabolism disorders can be found in acromegaly: Hypercalciuria[emedicine.medscape.com]

    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.

    Causes

    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[emedicine.medscape.com]
    • 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.[msdvetmanual.com]
    • Acromegalic subjects may develop congestive heart failure , particularly when blood pressure becomes high.[britannica.com]
    • Cardiovascular signs include the presence of a heart murmur, hypertension, arrhythmia, and congestive heart failure associated with hypertrophic cardiomyopathy. 3 Neurologic disease associated with feline acromegaly is uncommon but can occur with large[veterinarymedicine.dvm360.com]
    • heart failure Pulmonary: [1] [2] [5] [6] Obstructive sleep apnea Macroglossia Upper airway obstruction Ventilatory dysfunction Upper airway obstruction Metabolic: [1] [2] [6] Insulin resistance Impaired glucose metabolism Diabetes mellitus Visceral:[physio-pedia.com]
    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[emedicine.medscape.com]
    • They may also experience headaches and can often suffer from diabetes, hypertension, and heart failure.[pfizer.com]
    • Complications from increased growth hormone levels include atherosclerosis, peripheral neuropathy, hypertension, hyperglycemia, airway obstruction, cardiomyopathy, and visceromegaly involving the salivary glands, liver, spleen, and kidneys.[medical-dictionary.thefreedictionary.com]
    • References: [1] [7] [8] Complications Complications lead to increased mortality Cardiovascular complications ( CHF , hypertension , arrhythmia, valvular disease, hypertrophy): the main cause of death Diabetes mellitus Sleep apnea Neoplastic disease (e.g[amboss.com]
    • Complications Hypertension, coronary heart disease, cardiac failure, cerebrovascular disease.[patient.info]

    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.

    Other symptoms

    Pituitary Adenoma
    • […] of pituitary adenomas.[els.net]
    • A pituitary adenoma can usually be seen on CT scan or MRI.[dermnetnz.org]
    • Pituitary adenoma The most common type of tumor is a non-cancerous, or benign, tumor in the pituitary gland, known as a pituitary adenoma.[medicalnewstoday.com]
    • Clinical science Abstract Acromegaly is a condition in which benign pituitary adenomas lead to an excess secretion of growth hormone ( GH ) and insulin-like growth factor 1 ( IGF-1 ) .[amboss.com]
    • The MRI below shows a Pituitary Adenoma, that when combined with a history of Acromegaly is pretty diagnostic for a Pituitary Adenoma.[theartofmed.wordpress.com]
    McCune-Albright Syndrome

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    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.



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    1. Acromegaly facial features, CC BY 2.0

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