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...
...Endocrine Society (ES) recommends measur...
...the measurement of IGF-1 in patients without...
...nds measuring serum IGF-1 to rule out ac...
...recommends against relying on the use of ran...
...n patients with elevated or equivocal se...
...ollowing biochemical diagnosis of...
...gests magnetic resonance imaging (MRI)...
...performing formal visual field testing...
Presentation and Management of Comorbidities and M...
...S suggests evaluating all patients p...
...S also recommends that such comorbidities be...
...s screening for colon neoplasia with colonosco...
...ests a thyroid ultrasound if there is palpabl...
ES recommends assessing for hypopituitarism an...
Treatment
...eatment...
...als of Managem...
...suggests a biochemical target goal of an age-no...
...sts using a random GH...
...ests maintaining the same GH and IGF-1...
...urger...
...ndicatio...
...recommends transsphenoidal surgery as t...
...ests that repeat surgery be considered in a p...
...rative Medical Therapy...
ES suggests against routinely using pr...
...atients with severe pharyngeal thickness and...
...al Debulking...
...patient with parasellar disease making...
...operative Testing...
...y, ES suggests measuring an IGF-1 level and...
ES also suggests measuring a nadir GH level after...
...s performing an imaging study ≥12 weeks followi...
...ests MRI as the imaging modality of choice follow...
...erapeutic Options: Medica...
...medical therapy in a patient with persistent dis...
...ith significant disease (ie, with modera...
...patient with only modest elevations of serum IG...
...against routine abdominal ultrasound to monit...
...d should be performed if the patient has...
...rial imaging with MRI scan to evaluate tumor siz...
...uggests monitoring liver function tests month...
...suggests addition of pegvisomant or cabergoline i...
...use of a SRL as primary therapy in a pati...
...therapy/Radiosurgery...
...se of radiation therapy (RT) in the set...
...sts use of stereotactic radiotherapy...
...e efficacy of RT, ES recommends annual GH/IGF-1 re...
Following RT, ES recommends annual hormonal...
...l Circumstances...
...antism...
...ith the rare presentation of gigantism,...
Pregnan...
...women discontinue long-acting SRL formulations...
During pregnancy, ES recommends tha...
...egnancy, ES suggests serial visual field test...
...ests against monitoring GH and/or IGF-1 leve...
...sed for AcromegalyHaving trouble view...
...nagement of Acromegaly...