Last reviewed: 28 Sep 2021
Last updated: 19 Oct 2021

Summary

Definition

History and exam

Key diagnostic factors

  • coarsening of facial features
  • soft-tissue and skin changes
  • carpal tunnel syndrome
  • joint pain and dysfunction
  • snoring
  • alterations in sexual functioning
  • history or family history of inherited syndrome

Other diagnostic factors

  • fatigue
  • hypertension, arrhythmias
  • organomegaly
  • increased appetite, polyuria/polydipsia
  • headaches
  • visual field defects
  • signs and symptoms of hypopituitarism
  • cranial nerve palsies (e.g., ophthalmoplegia)

Risk factors

  • GPR101 over-expression
  • multiple endocrine neoplasia type 1 syndrome
  • isolated familial acromegaly
  • McCune-Albright syndrome
  • Carney complex

Diagnostic investigations

1st investigations to order

  • serum insulin-like growth factor 1 (IGF-1)
  • oral glucose tolerance test (OGTT)
  • random serum growth hormone (GH)

Investigations to consider

  • pituitary MRI or CT scan
  • GH-releasing hormone
  • chest and/or abdominal CT scanning
  • total body scintigraphy with radio-labelled somatostatin analogue (octreoscan)
  • PET scan with radio-labelled somatostatin analogue (Gallium-68 DOTATATE)
  • plasma cortisol
  • prolactin
  • thyroid-stimulating hormone (TSH) and free thyroxine
  • estradiol or testosterone
  • visual field testing

Treatment algorithm

Contributors

Authors

Maria Fleseriu, MD, FACE

Professor of Medicine (Endocrinology) and Neurological Surgery

Director

Pituitary Center

Oregon Health & Science University

Portland

OR

Disclosures

MF declares that she receives research funding (paid to Oregon Health & Science University) as well as scientific consulting fees from Chiasma, Crinetics, Ionis and Recordati; she receives scientific consulting fees from Ipsen and Pfizer. MF is an author of a number of references cited in this topic.

Acknowledgements

Professor Fleseriu would like to gratefully acknowledge Professor Ariel Barkan, Dr Omar Serri and Dr Sophie Vallette, previous contributors to this topic.

Disclosures

AB has received lecturing fees and research support from Novartis and Ipsen and is an author of several references cited in this topic. OS has received lecturing and educational program fees from Novartis Canada and is an author of a reference cited in this topic. SV has received fees for lecturing and attending a symposium and is an author of a reference cited in this topic.

Peer reviewers

Robert D. Murray, MBBS, BSc, FRCP, MD

Consultant Endocrinologist & Honorary Senior Lecturer

Department of Endocrinology

Leeds Centre for Diabetes & Endocrinology

Leeds Teaching Hospitals NHS Trust

St James’s University Hospital

Leeds

UK

Disclosures

RDM declares that he has no competing interests.

Shlomo Melmed, MD

Senior Vice President and Dean

Cedars Sinai Medical Center

Los Angeles

CA

Disclosures

SM has research grants of over 6 figures USD. SM is an author of a number of references cited in this topic.

Laurence Kennedy, MD, FRCP

Professor and Chief

Chairman

Department of Endocrinology, Diabetes and Metabolism

Cleveland Clinic

Cleveland

OH

Disclosures

LK declares that he has no competing interests.

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