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[Macronodular adrenal hyperplasia: a case report].

A Benchekroun1, Y Nouini, M Zannoud

  • 1Clinique urologique A, centre hospitalier universitaire Avicenne, Rabat, Maroc.

Annales D'Urologie
|May 14, 2003
PubMed
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This case report details a rare instance of Adrenocorticotropic Hormone (ACTH) independent bilateral adrenocortical macronodular hyperplasia (AIMAH) causing Cushing's syndrome. Treatment involved bilateral adrenalectomy due to the condition's resistance to standard therapies.

Area of Science:

  • Endocrinology
  • Rare diseases
  • Adrenal gland disorders

Background:

  • Cushing's syndrome can arise from various causes, including rare adrenal pathologies.
  • Adrenocorticotropic Hormone (ACTH) independent bilateral adrenocortical macronodular hyperplasia (AIMAH) is an uncommon condition.
  • Understanding AIMAH is crucial for diagnosing and managing complex Cushing's syndrome cases.

Observation:

  • A 53-year-old male presented with Cushing's syndrome.
  • The patient exhibited loss of diurnal cortisol rhythm and dexamethasone non-suppression.
  • Plasma ACTH levels were undetectable and unresponsive to corticotrophin-releasing hormone stimulation.

Findings:

  • The patient was diagnosed with ACTH-independent bilateral adrenocortical macronodular hyperplasia (AIMAH).

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  • This diagnosis was confirmed by hormonal assays showing cortisol autonomy.
  • The underlying aetiology of this specific case of AIMAH remained undetermined.

    • Implications:

    • This case highlights the diagnostic challenges posed by rare adrenal hyperplasia.
    • Bilateral adrenalectomy is presented as a definitive treatment for severe, refractory AIMAH.
    • Further research into the aetiology of AIMAH is warranted to explore potential targeted therapies.