Acromegaly
Key Points
Key Points
- Acromegaly is a chronic disorder caused by growth hormone (GH) hypersecretion.
- Over 95% of patients with acromegaly harbor a GH-secreting pituitary adenoma arising from somatotroph cells.
- In less than 5% of cases, excess growth hormone-releasing hormone (GHRH) secretion from a hypothalamic tumor or a neuroendocrine tumor (usually from lung or pancreas origin) may lead to somatotroph hyperplasia and acromegaly.
- More rarely, ectopic GH production by an abdominal or hematopoietic tumor may cause acromegaly.
- Hereditary conditions include multiple endocrine neoplasia-1 (MEN1), Carney complex, and McCune-Albright syndrome. Germline aryl hydrocarbon receptor interacting protein (AIP) mutations have been described in familial acromegaly with more aggressive tumors.
- Hypersecretion of GH leads to excess production of insulin-like growth factor-1 (IGF-1), leading to a multisystem disease characterized by somatic overgrowth, multiple comorbidities, premature mortality, and physical disfigurement.
- A multidisciplinary approach is critical for the management of acromegaly.
Diagnosis
Diagno...
The Endocrine Society (ES) recommends measurement...
...suggests the measurement of IGF-1 in...
...measuring serum IGF-1 to rule out acromegaly in a...
...recommends against relying on the...
In patients with elevated or equivocal serum I...
...chemical diagnosis of acromegaly, ES recommend...
...ts magnetic resonance imaging (MRI) as the...
...suggests performing formal visual field testing...
...Management of Comorbidities and M...
...ests evaluating all patients prese...
...nds that such comorbidities be lon...
...gests screening for colon neoplasia with...
...yroid ultrasound if there is palpable...
...recommends assessing for hypopituitarism and repl...
Treatment
...reatmen...
...als of Management...
...s a biochemical target goal of an age-norm...
...ests using a random GH...
...ggests maintaining the same GH and...
...urger...
...ndication...
...transsphenoidal surgery as the primary th...
...s that repeat surgery be considere...
...reoperative Medical...
...gests against routinely using preoperative medica...
...s with severe pharyngeal thickness and sleep apn...
...al Debulking...
...with parasellar disease making total...
...stoperative Te...
...g surgery, ES suggests measuring an IGF-1 lev...
...so suggests measuring a nadir GH level...
...mends performing an imaging study â...
...sts MRI as the imaging modality of c...
...eutic Options: Medical Therap...
...edical therapy in a patient with persistent d...
...h significant disease (ie, with moderate-to-sev...
...with only modest elevations of ser...
...ggests against routine abdominal ultrasound to m...
...nd should be performed if the patient has sign...
...erial imaging with MRI scan to evaluate tumor si...
ES suggests monitoring liver function...
...tion of pegvisomant or cabergoline in a patie...
...ggests use of a SRL as primary the...
...otherapy/Radiosurgery...
...use of radiation therapy (RT) in the setting of...
ES suggests use of stereotactic radiotherapy (SRT)...
...fficacy of RT, ES recommends annual GH/IGF...
...ES recommends annual hormonal testing of patient...
...pecial Circumsta...
...igantis...
...with the rare presentation of gigantism, ES r...
Pregnanc...
...ggests women discontinue long-acting SRL formul...
...ncy, ES recommends that acromegaly medical t...
...y, ES suggests serial visual field testing...
...uggests against monitoring GH and/or IGF-1 le...
...gs Used for AcromegalyHaving troubl...
Management of Acromega...