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
...iagnosis...
...Endocrine Society (ES) recommends mea...
...sts the measurement of IGF-1 in patie...
...ends measuring serum IGF-1 to rule out acrom...
...ends against relying on the use of random...
...ients with elevated or equivocal serum I...
...ollowing biochemical diagnosis of acromeg...
...magnetic resonance imaging (MRI) as the...
...ts performing formal visual field te...
...ation and Management of Comorbidities and Morta...
ES suggests evaluating all patients pr...
...also recommends that such comorbidities be longit...
...eening for colon neoplasia with colonoscopy...
ES suggests a thyroid ultrasound if there is...
...assessing for hypopituitarism and replacing h...
Treatment
...atment...
...of Management...
...a biochemical target goal of an age-normalized...
...suggests using a random GH...
...sts maintaining the same GH and IGF-1 assay in the...
Surgery
...ications...
...ranssphenoidal surgery as the primary therap...
...hat repeat surgery be considered in a patien...
...operative Medical Th...
...suggests against routinely using preoperative me...
...with severe pharyngeal thickness and...
...al Debulking...
...nt with parasellar disease making to...
...rative Testing...
...urgery, ES suggests measuring an IGF-1 level and a...
...lso suggests measuring a nadir GH lev...
...erforming an imaging study ≥12 weeks followin...
...uggests MRI as the imaging modality of choice...
Therapeutic Options: Medica...
...s medical therapy in a patient wit...
In a patient with significant dise...
...tient with only modest elevations of serum...
...inst routine abdominal ultrasound t...
...ltrasound should be performed if the patient...
...s serial imaging with MRI scan to e...
...sts monitoring liver function tests month...
...addition of pegvisomant or cabergoline in a pati...
...use of a SRL as primary therapy i...
...erapy/Radiosurgery...
...of radiation therapy (RT) in the...
...S suggests use of stereotactic radiotherapy (...
...o monitor the efficacy of RT, ES recomme...
Following RT, ES recommends annual ho...
...ial Circumstances...
...antism...
...patients with the rare presentation...
...gnancy...
ES suggests women discontinue long...
...cy, ES recommends that acromegaly medical t...
...g pregnancy, ES suggests serial visu...
...gests against monitoring GH and/or...
...gs Used for AcromegalyHaving trouble viewin...
...anagement of Acromegal...