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
...iagnosi...
The Endocrine Society (ES) recommends me...
...measurement of IGF-1 in patients without t...
...mends measuring serum IGF-1 to rule...
...ommends against relying on the use of random GH l...
...ts with elevated or equivocal serum I...
...emical diagnosis of acromegaly, ES reco...
ES suggests magnetic resonance imaging (MRI) a...
...ts performing formal visual field tes...
...nd Management of Comorbidities and Mortality Ris...
...aluating all patients presenting wit...
...mends that such comorbidities be longitud...
...reening for colon neoplasia with colon...
...ests a thyroid ultrasound if there is palp...
...recommends assessing for hypopituita...
Treatment
...reatment...
Goals of Managemen...
ES suggests a biochemical target go...
...S suggests using a rando...
...ntaining the same GH and IGF-1 assay in the...
...rgery
...ications...
...ranssphenoidal surgery as the primary therapy in m...
...ggests that repeat surgery be considered in...
...rative Medical Ther...
...sts against routinely using preoperative...
...r patients with severe pharyngeal thickness and...
...urgical Debulk...
...t with parasellar disease making total s...
Postoperative Testi...
...llowing surgery, ES suggests measuring an IGF-1...
...gests measuring a nadir GH level after...
...mends performing an imaging study ≥...
...sts MRI as the imaging modality of choice followe...
...erapeutic Options: Medic...
...mmends medical therapy in a patient...
...with significant disease (ie, with moderat...
...n a patient with only modest elevation...
...against routine abdominal ultrasound to...
...ound should be performed if the pat...
...s serial imaging with MRI scan to evaluate t...
...nitoring liver function tests monthly for t...
...s addition of pegvisomant or caberg...
...sts use of a SRL as primary therapy in a...
...adiotherapy/Radio...
...of radiation therapy (RT) in the...
...s use of stereotactic radiotherapy (SRT)...
...itor the efficacy of RT, ES recommends annual G...
...S recommends annual hormonal testi...
...pecial Circumstance...
...antism...
...with the rare presentation of gigantis...
...gnancy
...ggests women discontinue long-acting SRL formulat...
...regnancy, ES recommends that acromegaly medical...
...regnancy, ES suggests serial visual field testi...
...s against monitoring GH and/or IGF-1 levels dur...
...sed for AcromegalyHaving trouble viewing ta...
Management of Acrom...