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Acromegaly

Background

Overview

Definition
Acromegaly is a chronic, debilitating disease caused by chronic GH hypersecretion, which results in chronic medical comorbidities, poor QoL and high mortality rates.
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Pathophysiology
Acromegaly is caused by excess GH from a pituitary adenoma (90%).
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Disease course
Chronic excess of GH and insulin-like growth factor 1 results in acromegaly, which causes clinical manifestations of soft-tissue overgrowth, bone and joint disease, impaired glucose tolerance and diabetes, hypertension and cardiovascular complications, gonadal dysfunction, sleep apnea, impaired respiratory function, and colonic neoplasms.
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Prognosis and risk of recurrence
The mortality rate in untreated acromegaly is two to three times higher than that for the general population.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of acromegaly are prepared by our editorial team based on guidelines from the European Society of Endocrinology (ESE 2021), the Pituitary Society (PS 2021), the Endocrine Society (ES 2020,2014), the Acromegaly Consensus Conference (ACC 2018), and the European Thyroid Association (ETA 2018).
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Classification and risk stratification

Prognostic factors
As per PS 2021 guidelines:
Use biochemical control as the strongest predictor of patient outcomes, reflecting improvements in glucose metabolism, obstructive sleep apnea, CVD, and vertebral fractures, recognizing that structural heart and joint changes are unlikely to resolve.
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Do not use patient age as a predictor of surgical outcomes, recognizing that it does not impact the favorable effects of postsurgical remission on alleviating disease comorbidities.
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Diagnostic investigations

IGF-1: as per ES 2014 guidelines, obtain IGF-1 level measurement to rule out acromegaly in patients with a pituitary mass.
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  • GH

  • Brain imaging

  • Visual field testing

  • Evaluation of thyroid abnormalities

  • Screening for vertebral fractures

  • Screening for comorbidities

  • Screening for cancer

Medical management

Setting of care: as per ACC 2018 guidelines, manage patients with acromegaly at pituitary tumor centers of excellence to ensure the best and most cost-effective care.
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  • Indications for medical therapy

  • Therapeutic targets

  • Somatostatin receptor ligands

  • GH receptor antagonists

  • Combination therapy

  • Dopamine agonists

  • Temozolomide

  • Pituitary hormone replacement

  • Management of comorbidities

Therapeutic procedures

Radiotherapy: as per ES 2014 guidelines, consider offering radiotherapy in patients with residual tumor mass following surgery, or if medical therapy is unavailable, unsuccessful, or not tolerated.
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Perioperative care

Preoperative somatostatin receptor ligands: as per ACC 2018 guidelines, avoid administering somatostatin receptor ligands preoperatively to reduce the tumor size and improve surgical cure rates in patients with macroadenomas.
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Surgical interventions

Surgical resection: as per ACC 2018 guidelines, perform surgical resection of the pituitary adenoma, where possible, by an experienced neurosurgeon as the primary treatment for acromegaly.
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  • Surgical debulking

Specific circumstances

Pregnant patients, preconception care: as per ESE 2021 guidelines, manage female patients of reproductive age contemplating pregnancy with a diagnosis of pituitary adenoma, functioning or nonfunctioning, by an endocrinologist.
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  • Pregnant patients (evaluation)

  • Pregnant patients (management)

  • Pregnant patients (monitoring)

  • Pregnant patients (delivery and breastfeeding)

  • Patients with gigantism

Follow-up and surveillance

Laboratory follow-up, after surgery: as per PS 2021 guidelines, consider obtaining IGF-1 level measurement 6 weeks after surgery to assess remission in most patients with acromegaly, recognizing that patients with mildly elevated IGF-1 may yet normalize by 3-6 months.
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  • Laboratory follow-up (after radiotherapy)

  • Imaging follow-up (after surgery)

  • Imaging follow-up (on medical therapy)

  • Management of postoperative residual disease (repeat surgery)

  • Management of postoperative residual disease (radiotherapy)

  • Management of incomplete treatment response