ACTH-independent macronodular adrenal hyperplasia

Synonyms

7

Overview

Adrenocorticotropin independent macronodular adrenocortical hyperplasia (AIMAH) is considered a rare form of macronodular adrenal hyperplasia. It is an uncommon cause of primary adrenal hypercortisolism. In the literature that it is also mention a highly rare cause of Cushing syndrome. 

The prevalance of Cushing is estimated at 1/26,000 and ACTH is estimated to represent less than 1% of those cases. 

Symptoms

  • Nodular enlargement of both adrenal glands (multiple nodules of greater than 1cm)
  • Excess production of cortisol
  • Features of adrenocorticotropic hormone (ACTH) independent Cushing Syndrome
  • Hypercortisolism
  • Round face
  • Thin skin
  • Truncal obesity

Causes

Sporadic autosomal dominant 

Treatment

Bilateral adrenalectomy is considered the treatment of choice, with subsequent lifetime steroid replacement, but unilateral adrenalectomy has been recently proposed to reduce the secreting tissue. 

Resources

  • NIH