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.
Subject(s)
Female , Humans , Middle Aged , Abdomen , Adenoma , Adrenal Glands , Adrenalectomy , Adrenocorticotropic Hormone , Circadian Rhythm , Cushing Syndrome , Dexamethasone , Diabetes Mellitus , Hydrocortisone , Hyperplasia , Hypertension , Magnetic Resonance Imaging , Membranes , Pituitary Gland , Plasma , Tomography, X-Ray Computed , VasopressinsABSTRACT
Cushing's syndrome associated with nodular adrenal glands will be divided into four main categories: adrenal adenoma, adrenal carcinoma, primary pigmented nodular adrenal dysplasia (PPNAD) and macronodular adrenal hyperplasia(MAH). The term macronodular adrenal hyperplasia is restricted to the presence of multiple nodules visible to the naked eye, ranging in size from 0.5 to 7.0 cm. We report a case of Cushings syndrome caused by bilateral macronodular adrenal hyperplasia (MAH). A 45-year-old man presented with Cushingoid features, hypertension and diabetes mellitus. Urine free cortisol was 449.9 mmol/day(27-276) and were not suppressed after administration of low-dose and high-dose dexamethasone. Plasma ACTH was very low(1.87 pmol/L(18)) and was not stimulated by administration of ovine CRH. In abdominal CT, both adrenal glands were markedly enlarged and nodular in appearance. Pituitary MRI showed no abnormal finding. Bilateral adrenalectomy was done. Histologic examination revealed multiple nodules and internodular hyperplasia. This case and other reports suggested that because of variable biochemical, radiologic and pathologic findings, macronodular adrenal hyperplasia represents a heterogeneous group of patients with varying degrees of adrenal autonomy.