Preclinical Cushing's disease characterized by massive adrenal hyperplasia and hormonal changes after three years of metyrapone therapy.

Abstract

A 66-year-old woman had massive bilateral adrenal macronodular hyperplasia, found incidentally on an abdominal ultrasonogram. Her plasma ACTH and serum cortisol levels were normal, but they were not suppressed by low-dose dexamethasone. The patient did not exhibit any typical signs or symptoms of Cushing's disease. MRI showed no evidence of a tumor in the pituitary gland. A diagnosis of preclinical Cushing's disease was made, and she was treated with 11-hydroxylase inhibitor metyrapone. As the dose of metyrapone was increased, plasma ACTH levels gradually increased. After three years of treatment, she developed moon-face. Her plasma ACTH and serum cortisol concentrations were at their highest levels. A pituitary microadenoma was detected by MRI, whose source of ACTH was demonstrated by the definite step-up of central/peripheral ratio of ACTH obtained by cavernous sinus sampling. Overt Cushing's disease was diagnosed, and a pituitary tumor was removed by transsphenoidal surgery. In conclusion, the clinically and endocrinologically overt Cushing's disease characterized by macronodular adrenal hyperplasia was converted from a preclinical form. This case offers some insight into the clinical and biological features of preclinical Cushing's disease.

• Authors: Nariko Ohmori, Kaoru Nomura, Kazue Ohmori, Kazue Takano
• Journal: Endocrine journal (Endocr J) Vol. 54 Issue 3 Pg. 391-7 (Jun 2007) ISSN: 0918-8959 [Print] Japan
• PMID: 17446657 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antimetabolites
• Adrenocorticotropic Hormone
• Hydrocortisone
• Metyrapone

Topics

• ACTH-Secreting Pituitary Adenoma (diagnosis, surgery)
• Adenoma (diagnosis, surgery)
• Adrenal Glands (pathology)
• Adrenocorticotropic Hormone (blood)
• Aged
• Antimetabolites (therapeutic use)
• Female
• Humans
• Hydrocortisone (blood)
• Hyperplasia (complications)
• Metyrapone (therapeutic use)
• Pituitary ACTH Hypersecretion (blood, diagnosis, drug therapy, etiology)