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
Diagnosis
...ociety (ES) recommends measurement of IGF-1 levels...
...the measurement of IGF-1 in patients w...
...S recommends measuring serum IGF-1 t...
...ds against relying on the use of rando...
...ts with elevated or equivocal serum...
...ollowing biochemical diagnosis of ac...
ES suggests magnetic resonance imaging (MRI)...
...forming formal visual field testing wh...
...ntation and Management of Comorbidities and Mort...
...suggests evaluating all patients presenting...
...ds that such comorbidities be longitud...
...creening for colon neoplasia with colo...
...thyroid ultrasound if there is palpable thyroid...
...nds assessing for hypopituitarism and replacing h...
Treatment
...eatmen...
Goals of Mana...
ES suggests a biochemical target goal of an age-no...
...S suggests using a random GH...
...uggests maintaining the same GH and IGF...
...rgery...
...ications...
...commends transsphenoidal surgery as the...
...sts that repeat surgery be considered in a...
...rative Medical Therapy...
...against routinely using preoperative medical th...
...ts with severe pharyngeal thickness an...
...al Debulking
...n a patient with parasellar disease ma...
...erative Testing...
...lowing surgery, ES suggests measuring an IGF-...
...gests measuring a nadir GH level af...
...s performing an imaging study ≥12...
...as the imaging modality of choice followed by C...
...apeutic Options: Medical Ther...
...edical therapy in a patient with persistent...
...ient with significant disease (ie, with...
...patient with only modest elevations of...
...S suggests against routine abdominal...
...d be performed if the patient has si...
...ts serial imaging with MRI scan to...
ES suggests monitoring liver functio...
...ts addition of pegvisomant or caber...
...suggests use of a SRL as primary therapy in a pat...
...diotherapy/Radios...
...gests use of radiation therapy (RT) in the setti...
...se of stereotactic radiotherapy (SRT) over con...
...nitor the efficacy of RT, ES recommends a...
...recommends annual hormonal testing o...
...al Circumstances...
...antism...
...ents with the rare presentation of gigantism, E...
...egnancy...
...sts women discontinue long-acting SRL f...
...ng pregnancy, ES recommends that acromegaly...
...uring pregnancy, ES suggests serial v...
...gests against monitoring GH and/or IGF-1 lev...
...Used for AcromegalyHaving trouble viewin...
...ent of Acromegaly...