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
...gnosis...
...ne Society (ES) recommends measurement of I...
...ggests the measurement of IGF-1 in patie...
...nds measuring serum IGF-1 to rule out acro...
...nds against relying on the use of rand...
...ents with elevated or equivocal serum...
...llowing biochemical diagnosis of acromegaly,...
...suggests magnetic resonance imaging (MRI) as the...
...suggests performing formal visual field test...
...on and Management of Comorbidities a...
...s evaluating all patients presenting with ac...
...nds that such comorbidities be lon...
ES suggests screening for colon neoplasia wi...
...ts a thyroid ultrasound if there is...
...mends assessing for hypopituitarism and replacing...
Treatment
...reatme...
...als of Management...
...a biochemical target goal of an age...
ES suggests using a rand...
...intaining the same GH and IGF-1 assay in the s...
...urger...
...ndication...
ES recommends transsphenoidal surgery...
ES suggests that repeat surgery be...
...eoperative Medical Therapy
...ainst routinely using preoperative m...
...ents with severe pharyngeal thickness and...
Surgical Debulki...
...atient with parasellar disease making tota...
...operative Testin...
Following surgery, ES suggests measuring an IGF-...
...S also suggests measuring a nadir GH l...
...mends performing an imaging study ≥12 weeks fol...
...I as the imaging modality of choice follow...
...peutic Options: Medical Therap...
...edical therapy in a patient with persistent diseas...
...a patient with significant disease (ie, with...
...h only modest elevations of serum IGF-1...
...ts against routine abdominal ultra...
...ltrasound should be performed if the patient has...
...gests serial imaging with MRI scan to evaluat...
...itoring liver function tests monthly for the firs...
...tion of pegvisomant or cabergoline in a patie...
...e of a SRL as primary therapy in a p...
...rapy/Radiosurgery...
...suggests use of radiation therapy...
...use of stereotactic radiotherapy (SRT) over co...
...he efficacy of RT, ES recommends annual GH/IG...
...ing RT, ES recommends annual hormonal testi...
...Circumstances...
...antism...
...h the rare presentation of gigantism, E...
...regnancy
...n discontinue long-acting SRL formula...
..., ES recommends that acromegaly medical t...
...uring pregnancy, ES suggests serial visual...
...sts against monitoring GH and/or IG...
...rugs Used for AcromegalyHaving troubl...
...ent of Acromegaly...