Acromegaly

Publication Date: November 1, 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

...Diagn...

The Endocrine Society (ES) recommend...


...gests the measurement of IGF-1 in patients witho...


ES recommends measuring serum IGF-1 to rule...


...against relying on the use of random GH leve...


...nts with elevated or equivocal serum IGF...


...emical diagnosis of acromegaly, ES recommends...

...s magnetic resonance imaging (MRI) as the imaging...


...performing formal visual field testing when...


...Presentation and Ma...

...evaluating all patients presenting with ac...

...recommends that such comorbidities be longitudin...

...screening for colon neoplasia with co...

ES suggests a thyroid ultrasound if there...

...mends assessing for hypopituitarism and replac...


Treatment

Treatm...

Goals...

...suggests a biochemical target goal of...

...s using a random GH...

ES suggests maintaining the same GH and IGF-1...


...Indications...

...transsphenoidal surgery as the primary therapy in...

...S suggests that repeat surgery be consider...

...S suggests against routinely using preoperative...

...with severe pharyngeal thickness and sl...

...S...

...ient with parasellar disease makin...

...Postoperat...

...ollowing surgery, ES suggests measuring...

...also suggests measuring a nadir GH level aft...

...ds performing an imaging study ≥12 weeks followi...

...as the imaging modality of choice followed...


Therap...

...mmends medical therapy in a patient with...

...t with significant disease (ie, with moderate-...

...with only modest elevations of se...

...ests against routine abdominal ultrasound to...

...und should be performed if the patient has...

...ts serial imaging with MRI scan to evaluate tu...

...monitoring liver function tests monthly for the...

...gests addition of pegvisomant or cabergoli...

...of a SRL as primary therapy in a patient who can...


...Radiothera...

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

...suggests use of stereotactic radiothera...

...fficacy of RT, ES recommends annual...

...wing RT, ES recommends annual hormo...


...Special Circ...

...Gigantism...

...patients with the rare presentation of g...

...Pregnancy...

...gests women discontinue long-acting SRL...

...ng pregnancy, ES recommends that acromegal...

...ing pregnancy, ES suggests serial visual...

...suggests against monitoring GH and/or I...


...AcromegalyHaving trouble viewing table...


...ment of Acromegaly...