Corticotropin-independent Cushing's syndrome caused by an ectopic adrenal adenoma

Alejandro R Ayala, Shehzad Basaria, Robert Udelsman, William H. Westra, Gary S. Wand

Research output: Contribution to journalArticle

32 Citations (Scopus)

Abstract

Although nonsecreting suprarenal embryonic remnants are frequently found in the urogenital tract, adenomatous transformation resulting in glucocorticoid excess is a rare phenomenon. We report a case of a 63-yr-old woman that presented with new-onset hirsutism, facial plethora, hypertension, centripetal obesity, and a proximal myopathy. The 24-h urinary free cortisol excretion rate was elevated, and the serum ACTH level was suppressed. The patient failed an overnight and low dose dexamethasone suppression test and did not respond to CRH stimulation. In light of the undetectable baseline morning ACTH levels and the blunt response to CRH, the diagnosis of corticotropin-independent Cushing's syndrome was made. Imaging studies revealed normal adrenal glands and enlargement of a left pararenal nodule incidentally observed 4 yr before the onset of symptoms. Dramatic resolution of symptoms was observed after surgical removal of the 3.5-cm mass. Pathological exam confirmed adrenocortical adenoma in ectopic adrenal tissue. The case reported here represents the unusual circumstance in which the development of adenomatous transformation of ectopic adrenal tissue has been prospectively observed with imaging studies. It illustrates the importance of considering ectopic corticosteroid-secreting tumors in the context of corticotropin-independent Cushing's syndrome.

Original languageEnglish
Pages (from-to)2903-2906
Number of pages4
JournalJournal of Clinical Endocrinology and Metabolism
Volume85
Issue number8
DOIs
StatePublished - Dec 1 2000
Externally publishedYes

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Cushing Syndrome
Adenoma
Adrenocorticotropic Hormone
Choristoma
Adrenocortical Adenoma
Tissue
Imaging techniques
Hirsutism
Muscular Diseases
Adrenal Glands
Dexamethasone
Glucocorticoids
Hydrocortisone
Tumors
Adrenal Cortex Hormones
Obesity
Hypertension
Serum
Neoplasms

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology, Diabetes and Metabolism

Cite this

Corticotropin-independent Cushing's syndrome caused by an ectopic adrenal adenoma. / Ayala, Alejandro R; Basaria, Shehzad; Udelsman, Robert; Westra, William H.; Wand, Gary S.

In: Journal of Clinical Endocrinology and Metabolism, Vol. 85, No. 8, 01.12.2000, p. 2903-2906.

Research output: Contribution to journalArticle

Ayala, Alejandro R ; Basaria, Shehzad ; Udelsman, Robert ; Westra, William H. ; Wand, Gary S. / Corticotropin-independent Cushing's syndrome caused by an ectopic adrenal adenoma. In: Journal of Clinical Endocrinology and Metabolism. 2000 ; Vol. 85, No. 8. pp. 2903-2906.
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