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Cyclical Cushing's disease. A case report.

B Velkeniers1, A Beckers, A Stevenaert

  • 1Department of Endocrinology, University Hospital of Brussels, Belgium.

Pathology, Research and Practice
|June 1, 1991
PubMed
Summary
This summary is machine-generated.

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This case study details a patient with Cushing's syndrome and fluctuating adrenocorticotropic hormone (ACTH) hypersecretion. Despite extensive testing and treatment, the exact cause of ACTH excess remained elusive, suggesting a functional origin.

Area of Science:

  • Endocrinology
  • Neuroendocrinology
  • Oncology

Background:

  • Cushing's syndrome is a complex endocrine disorder caused by prolonged exposure to high cortisol levels.
  • Adrenocorticotropic hormone (ACTH) hypersecretion, often from the pituitary, is a common cause of Cushing's disease.
  • Intermittent or cyclical forms of Cushing's syndrome present diagnostic challenges.

Observation:

  • A 41-year-old man exhibited cyclical Cushing's syndrome with alternating remission and active phases of ACTH and cortisol hypersecretion.
  • Diagnostic tests, including metyrapone response and high-dose dexamethasone suppression, indicated a central origin of ACTH excess.
  • Inferior petrosal sinus sampling confirmed central ACTH hypersecretion.

Findings:

  • Near-total anterior hypophysectomy led to prolonged clinical remission.

Related Experiment Videos

  • Post-surgical pituitary examination revealed no identifiable corticotroph cell hyperplasia or microadenoma.
  • The findings challenge typical etiological classifications of Cushing's disease.

    • Implications:

    • This case suggests the possibility of functional ACTH hypersecretion, independent of a discrete pituitary adenoma or hyperplasia.
    • It highlights the importance of considering non-neoplastic causes for ACTH-dependent Cushing's syndrome.
    • Further research into the mechanisms of functional ACTH secretion is warranted.