Acromegaly

Publication Date: October 31, 2014

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...

...ocrine Society (ES) recommends meas...


...gests the measurement of IGF-1 in patients without...


...S recommends measuring serum IGF-1 to rule out ac...


...ainst relying on the use of random G...


...h elevated or equivocal serum IGF-1 levels,...


...chemical diagnosis of acromegaly, ES rec...

...sts magnetic resonance imaging (MRI) as the imagi...


...erforming formal visual field testing wh...


...d Management of Comorbidities and Mortality Ris...

...suggests evaluating all patients presenti...

...ends that such comorbidities be longitudinally mo...

...gests screening for colon neoplasia...

...suggests a thyroid ultrasound if t...

...ecommends assessing for hypopituitarism and...


Treatment

...atment

...s of Management...

...uggests a biochemical target goal of an ag...

...s using a random GH

...aintaining the same GH and IGF-1 assay in the sam...


...rgery...

...ndications...

...recommends transsphenoidal surgery as the prim...

...s that repeat surgery be considered in a patie...

...tive Medical Therapy...

...s against routinely using preoperative medica...

...patients with severe pharyngeal thicknes...

...cal Debulking...

...a patient with parasellar disease ma...

...perative Testing...

...lowing surgery, ES suggests measuring an...

...ests measuring a nadir GH level after a gl...

...performing an imaging study ≥12 weeks following...

...suggests MRI as the imaging modalit...


...rapeutic Options: Medical Th...

...mends medical therapy in a patient...

...patient with significant disease (...

...with only modest elevations of serum IGF-1...

...suggests against routine abdominal...

...hould be performed if the patient has s...

...al imaging with MRI scan to evaluate tumor...

...suggests monitoring liver function tests...

...gests addition of pegvisomant or cabergoline...

...gests use of a SRL as primary therapy in a...


...iotherapy/Radiosurgery...

...use of radiation therapy (RT) in the setting...

...suggests use of stereotactic radiothe...

...monitor the efficacy of RT, ES recommends annual...

Following RT, ES recommends annual hormonal tes...


...ial Circumstance...

...igantism...

...patients with the rare presentation of gigantism...

...gnancy

...ggests women discontinue long-acting SRL form...

...cy, ES recommends that acromegaly medical ther...

...uring pregnancy, ES suggests serial visual...

...ggests against monitoring GH and/or IGF-1 leve...


...sed for AcromegalyHaving trouble viewing table...


...nt of Acromegaly...