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.
Entire Body System
- Pain
[…] 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) High [medlineplus.gov]
Side effects may include loose stools, malabsorption, cholelithiasis (gall stones), local pain at the injection site. [acromegaly.org]
Easy fatigue Excessive sweating Decreased muscle strength (weakness) Limited joint mobility Joint pain Carpal tunnel syndrome Widely spaced teeth Swelling of the bony areas around a joint Additional symptoms that may be associated with this disease: [uclahealth.org]
These include: headache, described more often as "head pain" (due to dural tension) muscle pain, often misdiagnosed as fibromyalgia joint pain arthritis vertebral fractures with or without loss of bone mineral density carpal tunnel syndrome cardiovascular [radiopaedia.org]
[…] of discomfort or illness halos around lights itching skin joint pain lack of appetite loss of vision lower back or side pain muscle aches and pains night blindness overbright appearance of lights runny nose shivering sore throat stools that float, are [drugs.com]
- Fatigue
Most muscle function tests remained normal, but isometric flexion and the fatigue index were increased to 153 ± 42% and 139 ± 28% of predicted values, respectively. [ncbi.nlm.nih.gov]
There are symptoms such as fatigue, memory deficits, and there are a number of diseases associated with acromegaly. Please visit the Swedish Medical Center website for more information: http://www.swedish.org/Services/Neuro.... [youtube.com]
Decline in cortisol levels can cause weight loss, fatigue, low blood pressure etc. Excess secretion of other pituitary hormones like prolactin may be seen from the pituitary adenomas. Prolactin stimulates the breast to produce milk. [news-medical.net]
Side effects can include headaches, fatigue, and abnormal liver function. Dopamine agonists make up the third medication group. [niddk.nih.gov]
- Galactorrhea
In a series of 48 patients with galactorrhea, hyperprolactinemia and the presence of pituitary adenoma, eight patients (17%) had an increased GH level and clinical acromegaly ( 8 ). [edm.bioscientifica.com]
She did not complain of recent headaches, vomiting, visual difficulties, or galactorrhea and was clinically euthyroid. Her pulse rate was 84 beats/min, and her blood pressure was 150/90 mmHg. A visual field assessment did not reveal a defect. [ncbi.nlm.nih.gov]
Galactorrhea occurs in some women with acromegaly, usually in association with hyperprolactinemia. However, galactorrhea may occur with GH excess alone, because GH itself stimulates lactation. [merckmanuals.com]
[…] with pronounced under- or overbite, spreading teeth, and enlarging tongue Carpal tunnel syndrome Arthritis Oily skin and excessive sweating Tiredness, fatigue, depression Impotence, loss of libido Interrupted menstrual cycle (amenorrhea) Lactation (galactorrhea [acromegaly.org]
- Soft Tissue Swelling
Soft tissue swelling in the back of the throat can cause snoring and sleep apnea, leading to disturbed sleep and daytime sleepiness. [empoweryourhealth.org]
This leads to coarse facial features, soft tissue swelling (including the tongue), enlargement of the hands and feet, respiratory problems, hypertension, diabetes mellitus, carpal tunnel syndrome, and osteoarthritis. [ncbi.nlm.nih.gov]
Patients with acromegaly often suffer from headache, excessive sweating, soft-tissue swelling, joint disorders and, perhaps most striking, a progressive coarsening of facial features and enlargement of the hands, feet and jaw. [web.archive.org]
Lanreotide and octreotide also cause tumor shrinkage in 30-50% of patients, and improve soft tissue swelling, headache, joint pains and sleep apnea. [pacificneuroscienceinstitute.org]
Injections often improve symptoms of soft tissue swelling, headache, joint pains and sleep apnea. Patients require lifelong treatment with somatostatin. [pietromortini.com]
- Coarse Facial Features
This leads to coarse facial features, soft tissue swelling (including the tongue), enlargement of the hands and feet, respiratory problems, hypertension, diabetes mellitus, carpal tunnel syndrome, and osteoarthritis. [ncbi.nlm.nih.gov]
Coarse facial features are evident, including prognathism and prominence of the malar eminences and supraorbital ridges. By permission of the publisher. From Conrad C, Pro B, Prabhu S, et al. In Atlas of Cancer. Edited by M Markman and MR Gilbert. [msdmanuals.com]
Children with gigantism have few soft tissue effects (eg, peripheral edema, coarse facial features), because of their rapid linear growth. [emedicine.medscape.com]
Respiratoric
- Sleep Apnea
Enlarged bones in the nose and mouth may cause a large tongue and widely spaced teeth and may lead to sleep apnea. Bone and cartilage growth may lead to arthritis. [stanfordhealthcare.org]
apnea, and various other symptoms. [symptoma.com]
Sleep Apnea in Acromegaly Toraks Dernegi 16. [omicsonline.org]
Grunstein Endocrine and metabolic disturbances in obstructive sleep apnea Sleep and breathing, pp. 449-491 [18.] C. Laroche, G. Festal, S. [archbronconeumol.org]
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]
- Snoring
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]
Soft tissue swelling in the back of the throat can cause snoring and sleep apnea, leading to disturbed sleep and daytime sleepiness. [empoweryourhealth.org]
Symptoms and 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 [uvahealth.com]
- Hoarseness
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]
[…] in 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]
Symptoms: Enlarged hands Enlarged feet Widened fingers or toes due to skin overgrowth with swelling, redness, and pain Enlarged jaw (prognathism) and tongue Enlarged facial bones Thickening of the skin, skin tags Enlarged sebaceous glands Hoarseness Headache [uclahealth.org]
[…] 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 [nlm.nih.gov]
- Loud Snoring
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]
Symptoms of the disorder include wakefulness during the night, excessive sleepiness during the day, loud snoring, and/or obesity. [rarediseases.org]
Jaw & Teeth
- Prognathism
Craniofacial soft tissue and skeletal changes including mandibular prognathism and disturbed occlusion are typical manifestations of the disease process. [ncbi.nlm.nih.gov]
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]
Coarse facial features are evident, including prognathism and prominence of the malar eminences and supraorbital ridges. By permission of the publisher. From Conrad C, Pro B, Prabhu S, et al. In Atlas of Cancer. Edited by M Markman and MR Gilbert. [msdmanuals.com]
The mandible also characteristically enlarges resulting in prognathism and gaps between the teeth 10. This appearance is sometimes referred to as a "lantern jaw" 11 although this merely a descriptive term, not unique to acromegaly. [radiopaedia.org]
- Macroglossia
She was observed to have macroglossia, thickened coarse skin, acral enlargement, and newly detected, uncontrolled diabetes. A diagnosis of acromegaly was suspected. [ncbi.nlm.nih.gov]
Facies with prognathism and macroglossia, thick lips, prominent brow ridges (Figure 1), deep voice. Normal on auscultation. Normal fundoscopy. The patient reported no changes in body morphology. [revistanefrologia.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]
- Widely Spaced Teeth
Enlarged bones in the nose and mouth may cause a large tongue and widely spaced teeth and may lead to sleep apnea. Bone and cartilage growth may lead to arthritis. [stanfordhealthcare.org]
spaced teeth Large 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 [medlineplus.gov]
spaced teeth Swelling of the bony areas around a joint Additional symptoms that may be associated with this disease: Weight gain (unintentional) Hair, excessive on females Signs and tests: The level of growth hormone is high. [uclahealth.org]
Prognathism, widely spaced teeth, a large and furrowed tongue, large ears and elongated fingers are characteristic findings. Additional findings may include hirsutism in females, hypothyroidism and diabetes. Hypogonadism is frequent. [dermis.net]
Cardiovascular
- Hypertension
It causes overgrowth of body tissues and, if untreated, can lead to secondary problems such as hypertension and heart failure. Failure to seek medical treatment for this condition can reduce life expectancy by 10 years. [pfizer.com]
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 [symptoma.com]
Hypertension, diabetes and facial changes were significantly more frequent in those with AHI ≥15 events·h −1. [erj.ersjournals.com]
PowerPoint Download tables as PDFs Go to My Dashboard Close Pituitary tumor Acromegaly: excessive growth of hands, feet, jaw, and internal organs Gigantism if growth hormone (GH) excess before closure of epiphyses; acromegaly if after closure Amenorrhea, hypertension [accessmedicine.mhmedical.com]
- Heart Failure
It causes overgrowth of body tissues and, if untreated, can lead to secondary problems such as hypertension and heart failure. Failure to seek medical treatment for this condition can reduce life expectancy by 10 years. [pfizer.com]
We report a case of acromegaly in a 41 year old female patient who remained undiagnosed for 6 years and presented to us for the first time with symptoms of heart failure. [ncbi.nlm.nih.gov]
Most deaths result from heart failure, coronary artery disease and hypertension-related causes. [myvmc.com]
- Cardiomegaly
[…] teeth, and enlarging tongue Carpal tunnel syndrome Arthritis Oily skin and excessive sweating Tiredness, fatigue, depression Impotence, loss of libido Interrupted menstrual cycle (amenorrhea) Lactation (galactorrhea) Heart disease and heart enlargement (cardiomegaly [acromegaly.org]
Chest X-ray – may show cardiomegaly and osteoporosis. The rest of pituitary function should be investigated with measurement of prolactin, thyroid function, gonadotropins and sex steroids. [myvmc.com]
[…] and laryngeal tissue Deep, coarse voice May cause obstructive sleep apnea Goiter may be noted Hands Enlarged Fingers widen and rings no longer fit Carpal tunnel syndrome is common Feet grow, particularly as change in shoe width Hypertension (50%) and cardiomegaly [accessmedicine.mhmedical.com]
[…] include: headache, described more often as "head pain" (due to dural tension) muscle pain, often misdiagnosed as fibromyalgia joint pain arthritis vertebral fractures with or without loss of bone mineral density carpal tunnel syndrome cardiovascular cardiomegaly [radiopaedia.org]
- Chest Pain
pain, palpitations, SOB, previous MI etc. [medicaleducationleeds.com]
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]
Serious symptoms such as shortness of breath or chest pain warrant immediate attention. Exams and Tests Acromegaly is not an easy diagnosis, and it is often missed by health care providers. [emedicinehealth.com]
Eyes
- Visual Impairment
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]
Clinical presentation of acromegaly reported in an indian series ;vellore Clinical features • Male to female ratio • Mean age • Duration of symptoms • Head ache • Visual impairement • Prior apoplexy • Hypertension • Hypothyroidism • Hypocortisolism • [slideshare.net]
Musculoskeletal
- Arthritis
Bone and cartilage growth may lead to arthritis. People with acromegaly can also develop diabetes, high blood pressure, and other problems. The excess production of growth hormone usually is caused by a tumor of the pituitary gland. [stanfordhealthcare.org]
arthritic (acute) (chronic) (nonpyogenic) (subacute) M19.90 ICD-10-CM Diagnosis Code M19.90 Unspecified osteoarthritis, unspecified site 2016 2017 2018 2019 Billable/Specific Code Applicable To Arthrosis NOS Arthritis NOS Osteoarthritis NOS due to or [icd10data.com]
If not treated, acromegaly can lead to heart problems, arthritis, diabetes, vision problems, high blood pressure, or even death. [hopkinsmedicine.org]
Spontaneous rupture of flexor tendons within the carpal tunnel is rare in the absence of rheumatoid arthritis. [ncbi.nlm.nih.gov]
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 [symptoma.com]
- Arthralgia
Must be screened for in the presence of pituitary adenoma, profuse sweating, acral growth, coarsening of facial features, and when suspected in conjunction with commonly associated conditions, such as carpal tunnel syndrome, arthralgia, glucose intolerance [bestpractice.bmj.com]
The diagnosis of acromegaly, frequently made late because of the insidious nature of the disease, is generally based on symptoms of excess growth hormone, such as acral enlargement, soft-tissue swelling, arthralgia, jaw prognathism, hyperhidrosis, osteoarthritis [erj.ersjournals.com]
Common side effects of Somatuline® Depot include diarrhea, cholelithiasis, abdominal pain, nausea, injection site reactions, flatulence, arthralgia, and loose stools. [acromegaly.org]
[…] coarse voice May cause obstructive sleep apnea Goiter may be noted Hands Enlarged Fingers widen and rings no longer fit Carpal tunnel syndrome is common Feet grow, particularly as change in shoe width Hypertension (50%) and cardiomegaly Weight gain Arthralgias [accessmedicine.mhmedical.com]
Clinical features of acromegaly include soft tissue overgrowth, sweating, headaches and associated with hypertension, diabetes or glucose intolerance, arthralgia and sleep apnea syndrome: prolonged exposure to elevated levels of GH and IGF-I lead to both [ci.nii.ac.jp]
- Large Feet
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 [medicalnewstoday.com]
[…] bones of the face, large jaw and tongue, widely spaced teeth Large 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 [medlineplus.gov]
- Feet Enlargement
"Living with Acromegaly Reset the Balance Improve Your Outlook" View and print brochure Symptoms and signs may include: Soft tissue thickening of the palms of the hands and soles of the feet Enlargement of hands (ring size), feet (shoe size) and head [acromegaly.org]
- Joint Swelling
Arthritis, back pain, and curvature of the spine (kyphosis) - Due to enlargement of bones and cartilage in joints Swelling of the face, lips, and tongue Breathing problems during sleep - Due to narrowing of the airway resulting from swelling Tiredness [emedicinehealth.com]
Large joint arthropathy is a common feature of the disease, occurring in approximately 70% of patients, resulting from the thickening of cartilaginous and periarticular fibrous tissue, causing joint swelling, pain, and hypomobility followed by the narrowing [hindawi.com]
Skin
- Sweating
Must be screened for in the presence of pituitary adenoma, profuse sweating, acral growth, coarsening of facial features, and when suspected in conjunction with commonly associated conditions, such as carpal tunnel syndrome, arthralgia, glucose intolerance [bestpractice.bmj.com]
Because of night sweats, the selective serotonin reuptake inhibitors doses were modified. Night sweats persisted, and the patient was re-evaluated for other potential etiologies. [ncbi.nlm.nih.gov]
[…] and 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 [uvahealth.com]
Excessive sweating, deep voice and over-sized hands and feet are some of the symptoms associated with this disorder. [hospitals.jefferson.edu]
- Hyperhidrosis
Hyperhidrosis may also occur. Increased urinary secretion of calcium and phosphate is also associated with acromegaly. Measurement of: GH GHRH TRH Oral glucose IGF-1 Prolactin Imaging studies include MRI, CT scanning and radiography. [symptoma.com]
The diagnosis of acromegaly, frequently made late because of the insidious nature of the disease, is generally based on symptoms of excess growth hormone, such as acral enlargement, soft-tissue swelling, arthralgia, jaw prognathism, hyperhidrosis, osteoarthritis [erj.ersjournals.com]
[…] 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 ), excessive sweating ( hyperhidrosis [dermnetnz.org]
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]
- Hirsutism
Acromegaly also causes the Sign of Leser-Trelat (i.e., the eruption of multiple lesions of seborrheic keratosis). 1,2,6-9 Some cutaneous manifestations, such as hirsutism and sebaceous hypersecretion, may result from the free testosterone carrier protein [scielo.br]
[…] 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]
Additional findings may include hirsutism in females, hypothyroidism and diabetes. Hypogonadism is frequent. [dermis.net]
[…] 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 [nlm.nih.gov]
Others: prominent supraorbital ridge, hirsutism, thick greasy skin, carpal tunnel syndrome, visual field defects, galactorrhoea, hypertension, oedema, heart failure, arthropathy, proximal myopathy, glycosuria. [myvmc.com]
- Skin Thickening
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 [symptoma.com]
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]
(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]
(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 [nlm.nih.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]
- Hypertrichosis
Oily skin with large pores, hypertrichosis, and excessive sweating are common features. Pigmented skin tags, acanthosis nigricans, and psoriasis are also encountered. [ncbi.nlm.nih.gov]
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]
[…] 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]
Face, Head & Neck
- Frontal Bossing
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]
Markers Typically shows elevated levels of: growth hormone IGF-1 (insulin growth factor 1) Plain radiograph Skull Calvarial thickening, particularly of the inner table 10, frontal bossing, enlarged paranasal sinuses (especially frontal sinuses) and an [radiopaedia.org]
The forehead and overlying skin is thickened, sometimes leading to frontal bossing. There is a tendency towards mandibular overgrowth with prognathism, maxillary widening, tooth separation and jaw malocclusion. [orpha.net]
- Thick Lips
The facial aspect is characteristic and includes a widened and thickened nose, prominent cheekbones, forehead bulges, thick lips and marked facial lines. The forehead and overlying skin is thickened, sometimes leading to frontal bossing. [orpha.net]
Facies with prognathism and macroglossia, thick lips, prominent brow ridges (Figure 1), deep voice. Normal on auscultation. Normal fundoscopy. The patient reported no changes in body morphology. [revistanefrologia.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. Often people with acromegaly sweat a lot. [hormone.org]
- Nose Enlargement
Symptoms of acromegaly The main signs of acromegaly are: changes to facial features, such as a broadening nose, enlarging jaw and more widely spaced teeth enlarged lips or tongue enlarged hands and feet A pituitary tumour can also cause a range of other [healthdirect.gov.au]
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]
- Mandibular Prognathism
Craniofacial soft tissue and skeletal changes including mandibular prognathism and disturbed occlusion are typical manifestations of the disease process. [ncbi.nlm.nih.gov]
Facial changes include large lips and noses, frontal skull bossing and cranial ridges, mandibular overgrowth with prognathism, maxillary widening with teeth separation, jaw malocclusion, and overbite. [hindawi.com]
Urogenital
- Amenorrhea
Subsequently, she was referred to our university hospital for prolonged amenorrhea. [ncbi.nlm.nih.gov]
[…] figures into PowerPoint Download tables as PDFs Go to My Dashboard Close Pituitary tumor Acromegaly: excessive growth of hands, feet, jaw, and internal organs Gigantism if growth hormone (GH) excess before closure of epiphyses; acromegaly if after closure Amenorrhea [accessmedicine.mhmedical.com]
and jaw (prognathism) with pronounced under- or overbite, spreading teeth, and enlarging tongue Carpal tunnel syndrome Arthritis Oily skin and excessive sweating Tiredness, fatigue, depression Impotence, loss of libido Interrupted menstrual cycle (amenorrhea [acromegaly.org]
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]
Neurologic
- Headache
This highlights the use of monthly pasireotide in resolving headaches and improved biochemical control in a patient with acromegaly. [ncbi.nlm.nih.gov]
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. [symptoma.com]
They may also experience headaches and can often suffer from diabetes, hypertension, and heart failure. Who Gets Acromegaly and How? Only three or four patients per million individuals are diagnosed with the condition each year. [pfizer.com]
- Bitemporal Hemianopsia
In addition, bitemporal hemianopsia was observed on campimetry. After further examination, the patient was diagnosed with acromegaly. [ncbi.nlm.nih.gov]
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. [symptoma.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]
The general manifestations include neurological symptoms (headache, diplopia, bitemporal hemianopsia, carpal tunnel syndrome), osteoarthritis, visceromegalies, cardiomegaly, arterial hypertension, hypercalciuria and hoarseness (cartilaginous proliferation [scielo.br]
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.
X-Ray
- Nephrolithiasis
[…] vision, visual disturbance Uncommon (0.1% to 1%): Increased intraocular pressure [ Ref ] Genitourinary Common (1% to 10%): Urinary tract infection, pollakiuria Uncommon (0.1% to 1%): Gynecomastia, amenorrhea, polymenorrhea, oligomenorrhea, vaginitis, nephrolithiasis [drugs.com]
Reports indicate that hypercalciuria and nephrolithiasis may occur in 6–77% of patients with acromegaly. [hindawi.com]
• hypercalciuria 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 [slideshare.net]
Serum
- Hyperprolactinemia
The data support the hypothesis that hyperprolactinemia may have contributed to stimulating somatomedin C and sustaining the normal growth rate in this child. acromegaly growth hormone pituitary adenoma hyperprolactinemia Received December 31, 1987. [pediatrics.aappublications.org]
Medication-induced hyperprolactinemia and hypothyroidism were excluded. Initial head CT demonstrated a 2 mm pituitary microadenoma and a partial empty sella. [edm.bioscientifica.com]
Tumor damage to the pituitary stalk may cause hyperprolactinemia due to loss of inhibitory regulation of prolactin secretion by the hypothalamus. [emedicine.medscape.com]
CONCLUSION: The most important factors affecting gonadal functions, excluding central hypogonadism, are hyperprolactinemia and the duration of the indolent period before diagnosis of acromegaly. [ncbi.nlm.nih.gov]
- Hyperglycemia
Frequency of diabetes mellitus increased from 68% at 24 weeks to 77% at 48 weeks, and 9 patients discontinued PAS-LAR treatment, mainly because of severe hyperglycemia. [ncbi.nlm.nih.gov]
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]
[…] glucose-suppressed GH are usually complementary tests; however, disparities between GH and IGF-I levels occur in up to 30% of patients Prolactin may be elevated (cosecreted by many GH-secreting tumors) Insulin resistance usually present, often causing hyperglycemia [accessmedicine.mhmedical.com]
In Summary Commonly reported side effects of octreotide include: cardiac conduction disturbance, gallbladder sludge, and hyperglycemia. Other side effects include: cardiac arrhythmia, and hypoglycemia. [drugs.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]
Hypertension Cardiomyopathy Arrhythmias GI Colon polyps/cancer Neurologic Sleep apnea, both central and obstructive Narcolepsy Carpal tunnel syndrome Endocrine Insulin resistance/impaired glucose tolerance/impaired fasting glucose/diabetes mellitus Hypertriglyceridemia [unboundmedicine.com]
Complications Diabetes mellitus, hyperinsulinemia, and hypertriglyceridemia Airway narrowing, dyspnea, stridor and sleep apnea Hypertension, acromegalic heart, left ventricular hypertrophy Urolithiasis, hypercalciuria Muscle weakness, radiculopathies, [ncbi.nlm.nih.gov]
Colonoscopy
- Polyps
Among the 146 ACRO patients, 68% were subjected to at least one colonoscopy and in 32% of the cases a polyp was detected during the procedure. [ncbi.nlm.nih.gov]
However, to the best of our knowledge, the association of insulin-mediated pseudoacromegaly and adenomatous colonic polyps has not been previously reported. Adenomatous polyps are neoplastic polyps. [doi.org]
[…] increased spacing between front teeth Headaches Hoarse voice; sleep apnea and loud snoring Growth in hands and feet that requires larger shoes, rings, and gloves Appearance of skin tags and excessive sweating Arthritis of large joints and joint pain Polyps [pfizer.com]
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].
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.
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.
References
- 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.
- 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.
- 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.
- Ribeiro-Oliveira A Jr, Barkan A. The changing face of acromegaly--advances in diagnosis and treatment. Nat Rev Endocrinol 2012; 8:605.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- Fahlbusch R, Honegger J, Buchfelder M. Surgical management of acromegaly. Endocrinol Metab Clin North Am 1992; 21:669.
- 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.
- Sandret L, Maison P, Chanson P. Place of cabergoline in acromegaly: a meta-analysis. J Clin Endocrinol Metab. 2011 May. 96(5):1327-35.
- 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.