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Acromegaly

Acromegalia

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

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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.

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]
  • A 61-year-old man with obstructive sleep apnea syndrome and normal BMI complained of dyspnea. Nasofibroscopy revealed a global and major oedema of the glottis and supraglottis and also a paralysis of the left vocal fold.[ncbi.nlm.nih.gov]
  • Gordon Endoscopic findings in sleep apnea associated with acromegaly [22.] P. Chanson,J. Timsit,O.[archbronconeumol.org]
  • Acromegaly Growth Hormone Treatment Sleep Apnea Cardiovascular Diseases Bone Diseases Detailed Description: Acromegaly is a rare disease caused by a growth hormone (GH) producing pituitary adenoma.[clinicaltrials.gov]
  • The patient had been diagnosed with obstructive sleep apnea syndrome two years previously, since when he had worn a continuous positive airway pressure device during sleep.[ncbi.nlm.nih.gov]
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]
  • 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]
  • […] 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]
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]
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]
  • 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]
  • However, soft-tissue swelling occurs, and the peripheral nerves are enlarged. Delayed puberty or hypogonadotropic hypogonadism is also frequently present, resulting in a eunuchoid habitus.[merckmanuals.com]
Galactorrhea
  • 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]
  • 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]
  • […] hyperprolactinemia as well tend to have an earlier onset of disease, lesser acromegalic features, and lower GH levels, but also larger tumors. [22] Women with GH-prolactin–secreting adenomas tend to have higher incidences of menstrual disorders and galactorrhea[emedicine.medscape.com]
  • 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]
Coarse Facial Features
  • A 30-year-old Filipino man presented with a 11-year history of coarse facial features and progressive enlargement of hands and feet.[ncbi.nlm.nih.gov]
  • 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]
  • 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. Often people with acromegaly sweat a lot.[hormone.org]
Congestive Heart Failure
  • Its main features are biventricular hypertrophy, diastolic dysfunction, and in later stages, systolic dysfunction and congestive heart failure.[ncbi.nlm.nih.gov]
  • Acromegalic subjects may develop congestive heart failure , particularly when blood pressure becomes high. At times, excessive overgrowth of bone and cartilage involves the joints and causes pain.[britannica.com]
  • 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]
  • 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]
  • PubMed Google Scholar Bihan H, Espinosa C, Valdes-Socin H, Salenave S, Young J, Levasseur S, Assayag P, Beckers A, Chanson P: Long-term outcome of patients with acromegaly and congestive heart failure.[ojrd.biomedcentral.com]
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 skeleton elongation and mandibular overgrowth were combined with prognathism, malocclusion and overbite. Skull and CCJ alterations are of paramount importance when selecting the surgical approach, if surgery is indicated.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
Macroglossia
  • Inspection of the soft tissue revealed only macroglossia, although external palpation indicated a subcutaneous stiffness of the submandibular area.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
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]
  • 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]
  • 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]
  • 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]
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]
  • Acromegaly has several cardiovascular manifestations of which cardiomyopathy (CMP) and hypertension (HTN) are important and contribute to the increased mortality associated with the disease. Both these manifestations are reversible with treatment.[ncbi.nlm.nih.gov]
  • 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]
  • Hypertension is mentioned in the article as an important determinant of the rates of coexisting illness and death in patients with acromegaly. The pathogenesis of hypertension in acromegaly remains enigmatic and is probably multifactorial.[nejm.org]
Heart Failure
  • Its main features are biventricular hypertrophy, diastolic dysfunction, and in later stages, systolic dysfunction and congestive heart failure.[ncbi.nlm.nih.gov]
  • Here, we describe a case in which HTN and advanced heart failure resolved in an aged patient within a very short span of 2 months from the time of presentation.[ncbi.nlm.nih.gov]
  • 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]
  • Serious cardiovascular outcomes like stroke, heart failure and myocardial infarction were present in 5% at diagnosis. Erythrocyte levels were increased and correlated with IGF-1 values.[ncbi.nlm.nih.gov]
Cardiomegaly
  • […] 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]
  • […] swelling of soft tissue in the hands and feet (onset signs) enlarged bones in the skull, face, jaw, hands and feet joint pains pins and needles in the hands headaches gaps forming between the teeth, which may cause a ‘bad bite’ barrel chest enlarged heart (cardiomegaly[betterhealth.vic.gov.au]
  • 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]
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]
Arthritis
  • Spontaneous rupture of flexor tendons within the carpal tunnel is rare in the absence of rheumatoid arthritis.[ncbi.nlm.nih.gov]
  • If not treated, acromegaly can lead to heart problems, arthritis, diabetes, vision problems, high blood pressure, or even death.[cedars-sinai.edu]
  • 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]
Arthralgia
  • The most common AE was arthralgia (25.7%). Among 106 serious AEs reported by 42 patients, 10 were deemed related to therapy in 9 patients. At M24, 73.1% of patients rated LAN as convenient.[ncbi.nlm.nih.gov]
  • 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]
Large Feet
  • […] begins in childhood) Excessive sweating Headache Heart enlargement, which can cause fainting Hoarseness Joint pain , limited joint movement, swelling of the bony areas around a joint Large bones of the face, large jaw and tongue, widely spaced teeth Large[medlineplus.gov]
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]
  • 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]
  • 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]
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]
  • 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]
  • […] movement, swelling of the bony areas around a joint Large 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[medlineplus.gov]
  • 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]
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]
  • 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]
  • 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]
  • She had hirsutism and wide spaced teeth without other clinical features of acromegaly.[edm.bioscientifica.com]
Coarseness of the Skin
  • Symptoms may include: swelling of the hands and feet facial features become coarse as bones grow body hair becomes coarse as the skin thickens and/or darkens increased perspiration accompanied with body odor protruding jaw voice deepening enlarged lip[hopkinsmedicine.org]
  • 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]
  • 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]
Hyperpigmentation
  • […] 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]
  • […] 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]
  • […] no conclusive studies exist to substantiate this finding Hypertrichosis (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[emedicine.medscape.com]
  • Hyperpigmentation occurs in roughly 40% of cases and almost always in photoexposed areas. It is probably due to the associated increase of melanotrophic hormone. Acanthosis negricans occurs in 10% of cases. Hypertrophic scarring is common.[scielo.br]
  • […] 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]
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]
  • 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]
  • 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]
  • 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]
  • . • Obstruction of the upper airways is a result of macroglossia, prognathism, thick lips, and hypertrophy of the laryngeal mucosa and cartilage; • it can cause sleep apnea and excessive snoring and can complicate tracheal intubation during anesthesia[slideshare.net]
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]
Headache
  • This highlights the use of monthly pasireotide in resolving headaches and improved biochemical control in a patient with acromegaly.[ncbi.nlm.nih.gov]
  • Furthermore, unrelated analgesic and biochemical effects of SSAs supported a complex pathogenesis of acromegaly-associated headache.[ncbi.nlm.nih.gov]
  • […] one sees atypical headache presentations.[nejm.org]
  • A young man with subtle clinical features suggestive of hypersomatotropism presented with acute-onset severe headache. Relevant investigations confirmed polycythaemia and growth hormone (GH)-secreting pituitary macroadenoma with apoplexy.[ncbi.nlm.nih.gov]
  • A high index of suspicion for incipient meningitis should be maintained when patients present with severe headache and increased intracranial pressure, even if they initially lack the typical symptoms and signs.[ncbi.nlm.nih.gov]
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]
  • 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.[pietromortini.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]
Amenorrhea
  • Subsequently, she was referred to our university hospital for prolonged amenorrhea.[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]
  • 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]
  • ) 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]

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.

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]
  • 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]
  • Logistic regression analysis revealed that medications, age, GH level, or IGF-1 level before medication, hyperprolactinemia, and prior gamma-knife surgery or radiation therapy, did not affect the therapeutic response.[ncbi.nlm.nih.gov]
  • 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]
  • Tumor damage to the pituitary stalk may cause hyperprolactinemia due to loss of inhibitory regulation of prolactin secretion by the hypothalamus.[emedicine.medscape.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]
Polyps
  • The general risk of polyps and adenomatous polyps in ACRO patients was higher compared to the control population of Veneto Region, Italy (odds ratio 1.33 and 1.16, respectively). No cancerous polyps were detected in our analysis.[ncbi.nlm.nih.gov]
  • Twelve patients had adenoma (41.4%), eight patients had hyperplastic polyps (27.6%), three patients had sessile serrated adenoma/polyps (10.3%), and three patients had colon cancer (10.3%).[ncbi.nlm.nih.gov]
  • Benign neoplasms were found in majority of patients with acromegaly (71.6%) most notably: nodular goiter and colon polyps; malignant lesions were rare (4.5%). Only every fifth patient suffered from no neoplastic proliferations.[ncbi.nlm.nih.gov]
  • Thyroid nodules were frequent (34.0%); 820 patients had colonoscopy at diagnosis and 13% had polyps. Osteoporosis was present at diagnosis in 12.3% and 0.6-4.4% had experienced a fracture.[ncbi.nlm.nih.gov]
  • Internally, acromegaly causes organs to enlarge and can contribute to the development of diabetes, heart disease, and even colon polyps.[weillcornellbrainandspine.org]

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.

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.

References

Article

  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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Last updated: 2017-08-09 17:55