Preclinical Cushing's disease characterized by massive adrenal hyperplasia and hormonal changes after three years of metyrapone therapy.
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.
Ohmori
Nariko
N
Department of Medicine, Institute of Clinical Endocrinology, Tokyo, Japan.
Nomura
Kaoru
K
Ohmori
Kazue
K
Takano
Kazue
K
D049913:ACTH-Secreting Pituitary Adenoma / Q000175:diagnosis / Q000601:surgery; D000236:Adenoma / Q000175:diagnosis / Q000601:surgery; D000311:Adrenal Glands / Q000473:pathology*; D000324:Adrenocorticotropic Hormone / Q000097:blood*; D000368:Aged; D000963:Antimetabolites / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood*; D006965:Hyperplasia / Q000150:complications; D008797:Metyrapone / Q000627:therapeutic use*; D047748:Pituitary ACTH Hypersecretion / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology*
D000963:Antimetabolites; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone; D008797:Metyrapone