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
...Dia...
...Endocrine Society (ES) recommends meas...
...suggests the measurement of IGF-1 in patients...
...s measuring serum IGF-1 to rule out acrome...
...S recommends against relying on the use of rando...
...nts with elevated or equivocal serum IGF-...
...ollowing biochemical diagnosis of acromegaly...
...etic resonance imaging (MRI) as the imag...
...gests performing formal visual field testing wh...
...Presentat...
...s evaluating all patients presenting with...
...mmends that such comorbidities be longitudin...
...S suggests screening for colon neoplasia with c...
...hyroid ultrasound if there is palpable thyroid nod...
...S recommends assessing for hypopituit...
Treatment
...Treatment...
...Goals of M...
...iochemical target goal of an age-normalized...
...S suggests using a random...
...maintaining the same GH and IGF-1 assay in...
...Surgery...
...In...
...anssphenoidal surgery as the primary the...
...gests that repeat surgery be considered in...
...Preoperat...
...S suggests against routinely using preopera...
...ts with severe pharyngeal thickness and...
...Surgica...
...with parasellar disease making total surgical r...
...g surgery, ES suggests measuring an IGF-1 level...
...also suggests measuring a nadir GH lev...
...ends performing an imaging study ≥12...
...uggests MRI as the imaging modality...
...Therapeutic...
...medical therapy in a patient with persi...
...ient with significant disease (ie, with mo...
...with only modest elevations of serum...
...suggests against routine abdominal ultrasou...
...ltrasound should be performed if the pa...
...al imaging with MRI scan to evaluate tumor size in...
...gests monitoring liver function tests m...
...S suggests addition of pegvisomant or ca...
ES suggests use of a SRL as primary therapy in a p...
...Radiotherapy/R...
...s use of radiation therapy (RT) in the sett...
...ts use of stereotactic radiotherapy (SRT) ov...
...e efficacy of RT, ES recommends annu...
..., ES recommends annual hormonal testing of p...
...Special Circumstances...
...Gigantism
...s with the rare presentation of gigantism, ES...
...Pregnancy...
...women discontinue long-acting SRL formulation...
...uring pregnancy, ES recommends that acrome...
...nancy, ES suggests serial visual field tes...
...against monitoring GH and/or IGF-1...
...rugs Used for Acromegaly Drug (Bran...
...ment of Acromegaly...