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

...agnosis...

...e Endocrine Society (ES) recommends measureme...


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


...mends measuring serum IGF-1 to rule out acrom...


...ainst relying on the use of random GH le...


In patients with elevated or equivo...


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

...sts magnetic resonance imaging (MRI) as th...


...S suggests performing formal visual fiel...


...ation and Management of Comorbidities and Mor...

...ggests evaluating all patients pres...

ES also recommends that such comorbidit...

...sts screening for colon neoplasia with colonoscop...

...yroid ultrasound if there is palpable...

...S recommends assessing for hypopituitaris...


Treatment

...atment...

...ls of Managem...

...uggests a biochemical target goal of an age-normal...

...uggests using a random...

...gests maintaining the same GH and IGF-1 assay in...


...urgery...

...ndicatio...

...ommends transsphenoidal surgery as the prim...

...suggests that repeat surgery be cons...

Preoperative Medical Therap...

...ts against routinely using preoperative medical th...

...r patients with severe pharyngeal thickness and...

...al Debulking...

...patient with parasellar disease ma...

...ostoperative Te...

...urgery, ES suggests measuring an IGF-1 le...

...uggests measuring a nadir GH level after a glu...

...ommends performing an imaging study ≥12 weeks fo...

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


...eutic Options: Medical Therap...

...recommends medical therapy in a pa...

...patient with significant disease (ie, w...

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

...S suggests against routine abdominal ultrasound t...

Ultrasound should be performed if the pati...

...gests serial imaging with MRI scan to ev...

...ts monitoring liver function tests monthly for...

ES suggests addition of pegvisomant or c...

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


...diotherapy/Radios...

ES suggests use of radiation therapy (...

...suggests use of stereotactic radiotherapy...

...monitor the efficacy of RT, ES recommend...

...owing RT, ES recommends annual hormonal...


Special Circumstance...

...gantism...

...ients with the rare presentation of gi...

...regnanc...

ES suggests women discontinue long-...

...ncy, ES recommends that acromegaly medica...

...regnancy, ES suggests serial visual field testing...

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


...ed for AcromegalyHaving trouble viewing table?...


...anagement of Acr...