J Korean Soc Endocrinol.  2005 Aug;20(4):390-394. 10.3803/jkes.2005.20.4.390.

A Case of Bilateral Macronodular Adrenocortical Hyperplasia Accompanied by Hyperresponsiveness to Vasopressin

• Kim DI
• Lee SH
• Kim EA
• Park HL
• Lee GU
• Hong SB
• Nam MS
• Shin SH
• Kim YS

• KMID: 2100471
• DOI: http://doi.org/10.3803/jkes.2005.20.4.390

Abstract

Cushing's syndrome associated with nodular adrenal hyperplasia glands is divided into 4 main categories: adrenal adenoma, adrenal carcinoma, primary pigmented nodular adrenal dysplasia and macronodular adrenal hyperplasia(MAH). The mechanism of bilateral MAH, when ACTH is suppressed, was previously unknown, and referred to as being "autonomous". Recently, several reports have shown MAH to be under the control of ectopic or eutopic membrane hormone. Here, a case of Cushing's syndrome, caused by bilateral MAH, is reported. A 62-year-old woman presented with Cushingoid features, hypertension and diabetes mellitus. In her case, abnormal adrenal stimulation of cortisol secretion in response to exogenous vasopression stimulation was shown. Her urine free cortisol was 726.0microgram/dL, which was not suppressed after administration of high-dose dexamethasone. Her plasma cortisol level was elevated, but without circadian rhythm. ACTH was undetectable. An abdomen CT scan demonstrated bilaterally enlarged multinodular adrenal glands. A Sella MRI revealed no alteration of the pituitary gland. The patient underwent a laparoscopic bilateral adrenalectomy. Histological examination revealed bilateral macronodular hyperplasia. After having recovered, the patient showed progressive regression of the Cushingoid status.

MeSH Terms

Abdomen
Adenoma
Adrenal Glands
Adrenalectomy
Adrenocorticotropic Hormone
Circadian Rhythm
Cushing Syndrome
Dexamethasone
Diabetes Mellitus
Female
Humans
Hydrocortisone
Hyperplasia*
Hypertension
Magnetic Resonance Imaging
Membranes
Middle Aged
Pituitary Gland
Plasma
Tomography, X-Ray Computed
Vasopressins*
Adrenocorticotropic Hormone
Dexamethasone
Hydrocortisone
Vasopressins

Figure

• 

  Fig. 1 A) abdominal CT (with contrast) shows bilaterally enlarged multinodular adrenal glands. B) Brain MRI (T1 with Gd enhancement) shows no abnormalities of pituitary gland.

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  Fig. 2 A) & B) Gross appearance shows multinodular yellowish brown soft tissue. C) & D) Microscopic view (×10, C) (×200, D) revealed macronodular hyperplasia.

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