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
[…] 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 Joint pain [nlm.nih.gov]
[…] 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]
The most common side effects of SOMATULINE DEPOT in people with acromegaly include diarrhea; stomach (abdominal) pain; nausea; and pain, itching, or a lump at the injection site. SOMATULINE DEPOT may cause dizziness. [acromegaly.somatulinedepot.com]
[…] 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]
Side effects can include headaches, fatigue, and abnormal liver function. Dopamine agonists make up the third medication group. [niddk.nih.gov]
Symptoms of acromegaly may include any of the following: Body odor Blood in the stool Carpal tunnel syndrome Decreased muscle strength ( weakness ) Decreased peripheral vision Easy fatigue Excessive height (when excess GH production begins in childhood [nlm.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]
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]
- Soft Tissue Swelling
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]
Injections often improve symptoms of soft tissue swelling, headache, joint pains and sleep apnea. Patients require lifelong treatment with somatostatin. [pietromortini.com]
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]
Soft tissue swelling in the back of the throat can cause snoring and sleep apnea, leading to disturbed sleep and daytime sleepiness. [empoweryourhealth.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]
- Tall Stature
(jaw protrusion) Aromatase deficiency or estrogen receptor deficiency causing tall stature Other causes of increased GH level Exercise or eating prior to test Acute illness or agitation Liver failure or kidney disease Malnourishment Diabetes mellitus [accessmedicine.mhmedical.com]
Convert to ICD-10-CM : 253.0 converts approximately to: 2015/16 ICD-10-CM E22.0 Acromegaly and pituitary gigantism Approximate Synonyms Acromegaly Constitutional tall stature Growth hormone overproduction Hypersomatotropic gigantism Overproduction of [icd9data.com]
2018 2019 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 2019 Billable/Specific Code Applicable To Constitutional gigantism [icd10data.com]
Tall stature may result from hereditary, dietary, or other factors. Gigantism is caused by disease or disorder in those parts of the endocrine system that regulate growth and development. [britannica.com]
The same excess of growth hormone in individuals whose epiphyses have not fused will result in gigantism (excessively tall stature). [radiopaedia.org]
Respiratoric
- Sleep Apnea
The aim of the ACROSAHS study was to investigate the prevalence of acromegaly and acromegaly comorbidities in patients with sleep apnea symptoms and acral enlargement. [ncbi.nlm.nih.gov]
Grunstein Endocrine and metabolic disturbances in obstructive sleep apnea Sleep and breathing, pp. 449-491 [18.] C. Laroche, G. Festal, S. [archbronconeumol.org]
FDA-regulated Device Product: No Additional relevant MeSH terms: Cardiovascular Diseases Sleep Apnea Syndromes Acromegaly Bone Diseases Apnea Respiration Disorders Respiratory Tract Diseases Sleep Disorders, Intrinsic Dyssomnias Sleep Wake Disorders Nervous [clinicaltrials.gov]
Sleep Apnea in Acromegaly Toraks Dernegi 16. [omicsonline.org]
- Snoring
Soft tissue within the body can also enlarge, causing increased snoring, sleep apnea, chronic sinus problems, radiculopathy from nerve root entrapment (i.e., carpal tunnel syndrome), joint pain and stiffness, increase in sweating (hyperhydrosis), and [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]
He snored, but had never been evaluated for obstructive sleep apnea. He also experienced excessive sweating and mild fatigue and his lower jaw had enlarged over the years, increasing the spacing between his teeth. [brighamhealthhub.org]
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]
- 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 [nlm.nih.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]
- 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]
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. [merckmanuals.com]
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]
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
Inspection of the soft tissue revealed only macroglossia, although external palpation indicated a subcutaneous stiffness of the submandibular area. [ncbi.nlm.nih.gov]
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 [nlm.nih.gov]
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]
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]
Cardiovascular
- Hypertension
Increased mortality has been related to cardiovascular events that could be linked to these hormones and patients suffer from high rates of diabetes and hypertension. [ncbi.nlm.nih.gov]
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]
PowerPoint Download tables as PDFsGo 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
Its main features are biventricular hypertrophy, diastolic dysfunction, and in later stages, systolic dysfunction and congestive heart failure. [ncbi.nlm.nih.gov]
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]
- Cardiomegaly
[…] 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]
[…] 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 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]
Skin
- Sweating
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]
[…] 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]
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]
Excessive sweating, deep voice and over-sized hands and feet are some of the symptoms associated with this disorder. [hospitals.jefferson.edu]
- Hyperhidrosis
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]
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]
Headache, vision loss ( bitemporal hemianopsia ), cranial nerve palsies ♀ : Oligomenorrhea, secondary amenorrhea, galactorrhea, vaginal atrophy ♂ : Erectile dysfunction, decreased libido, ↓ testicular volume Soft tissue effects Doughy skin texture, hyperhidrosis [amboss.com]
[…] 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, degenerative arthritis, and spinal stenosis may occur Colon polyps common Skin Hyperhidrosis [accessmedicine.mhmedical.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 [nlm.nih.gov]
Additional findings may include hirsutism in females, hypothyroidism and diabetes. Hypogonadism is frequent. [dermis.net]
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]
- Skin Thickening
(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]
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]
(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]
- 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]
(found in approximately one half of acromegaly patients): Unlike virilizing disorders, hypertrichosis of acromegaly does not affect the beard area Oily skin (acne is not common) Hyperpigmentation (40% of patients) Acanthosis nigricans (a small percentage [emedicine.medscape.com]
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]
Musculoskeletal
- Arthritis
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]
Spontaneous rupture of flexor tendons within the carpal tunnel is rare in the absence of rheumatoid arthritis. [ncbi.nlm.nih.gov]
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]
If not treated, acromegaly can lead to heart problems, arthritis, diabetes, vision problems, high blood pressure, or even death. [cedars-sinai.edu]
- 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]
[…] 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]
Common side effects of Somatuline® Depot include diarrhea, cholelithiasis, abdominal pain, nausea, injection site reactions, flatulence, arthralgia, and loose stools. [acromegaly.org]
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
[…] 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 [nlm.nih.gov]
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]
- 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]
Face, Head & Neck
- Frontal Bossing
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]
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]
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]
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 PDFsGo 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]
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]
This can cause symptoms such as diminished sexual function, loss of menstrual periods (amenorrhea), and symptoms of an underactive thyroid gland. A pituitary problem diagnosed based upon these symptoms before the symptoms of acromegaly are apparent. [empr.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]
It has been established in a prospective study that the size of a pituitary tumor does not determine the headache presentation. 2 Moreover, the phenotype of headache presentations is wide, including migraine and the trigeminal autonomic cephalalgias, [nejm.org]
Our patient had been evaluated elsewhere for severe headaches which were accompanied by occasional blurred vision and sensitivity to light. [brighamhealthhub.org]
- Bitemporal Hemianopsia
In addition, bitemporal hemianopsia was observed on campimetry. After further examination, the patient was diagnosed with acromegaly. [ncbi.nlm.nih.gov]
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]
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]
Headache Superior Spread leads to compression of the Optic Chiasm leading to bitemporal hemianopsia. Inferior Spread leads to sphenoid sinus invasion. Lateral Spread leads to Cavernous Sinus invasion. [theartofmed.wordpress.com]
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]
The objective of this study was to investigate the effect of hyperprolactinemia and high levels of insulin-like growth factor-I (IGF-I) on bone resorption and their relation with receptor activator of nuclear factor-κB ligand (RANKL) and osteoprotegerin [ncbi.nlm.nih.gov]
- 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]
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]
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]
Kumar S, Durrington PN, O’Rahilly S, et al, 1996 Severe insulin resistance, diabetes mellitus, hypertriglyceridemia, and pseudoacromegaly. J Clin Endocrinol Metab 81: 3465-3468. 13. [doi.org]
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]
Colonic polyps. Vertebral fractures, possibly due to low-quality bone despite high bone mass. [patient.info]
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.