Cushing's Syndrome due to Bilateral Adrenocortical Adenomas with Different Pathological Features.

Abstract

A 48-year-old womanwith Cushing's syndrome due to bilateral adrenocortical adenomas is reported. The patient presented with a typical Cushingoid appearance. The serum cortisol level was elevated with loss of the diurnal rhythm and the plasma adrenocorticotropic hormone (ACTH) level was undetectable. Dynamic testing showed no suppression of urinary 17-OHCSby high-dose dexamethasone and no stimulation by metyrapone. An abdominal computed tomography (CT) scan showed bilateral adrenal tumors. Bilateral adrenalectomy was performed. The right adrenal gland contained a tumor that was encapsulated and consisted mainly of compact cells. The surrounding cortex was atrophic. The left adrenal gland contained an encapsulated tumor composedpredominantly of clear cells. There were numerous small adrenocortical nodules in the surrounding cortex. Immunohistochemical analysis of steroidogenic enzymes (P450scc, 3P-HSD, P450c21, P450cl7 and P450cll) was performed. Immunoreactivity of all the enzymes was intense in the compact cells of the right adrenocortical adenoma,while the adjacent non-neoplastic cortex was negative for the enzymes. In the left adrenal tumor, the immunoreactivity of 3(3-HSD was intense, while that of P450cl7 was weak. In the adrenocortical nodules, 3(3-HSD activity was sporadically observed. G protein genes encoding Gs a and Gi2 were examined for activating mutations at codons 201 and 227 (Gs a) and codons 179 and 205 (Gi2 a) in the bilateral adrenal tumors, but no mutations were found. The bilateral adenomas of this patient showed marked differences in microscopic and immunohistochemical studies, suggesting that the capacity of steroidogenesis differs between the right and left tumors. (Internal Medicine 36: 804-809, 1997)

Bilateral adrenocortical adenomas causing adrenocorticotropic hormone-independent Cushing’s syndrome: A case report and review of the literature

“…Some studies have revealed that steroidogenic enzymes, such as P450scc, 3β-HSD, P450c21, P450c17, and P450c11, are intensely expressed in the compact cells of the adenoma, while their expression profiles are negative in the adjacent non-neoplastic cortex. At the same time, the histological features may differ between two adenomas[14,15]. It is difficult to distinguish between functioning or nonfunctioning adrenal cortical masses by the imaging characteristics on CT scanning[1].…”

The Clinical Conundrum of Corticotropin‐independent Autonomous Cortisol Secretion in Patients with Bilateral Adrenal Masses

“…The imaging method employing [6β‐ 131 I]iodomethyl‐19‐norcholesterol (NP‐59) was introduced in the late 1970s [23] as a means of assessing adrenal cortical function. The NP‐59 scan, performed with dexamethasone suppression, had the advantage of correlating autonomous cortical function (typically primary aldosteronism) with anatomical abnormalities.…”

“…Although the diagnosis and management of corticotropin (ACTH)-independent Cushing syndrome (CS) or subclinical CS in patients with a unilateral cortical adenoma is relatively straightforward [1,2], the management of patients with bilateral adrenal masses in these settings is problematic [3][4][5][6][7][8][9][10][11][12]. These patients may have a unilateral cortisol-secreting adenoma with a contralateral nonfunctioning cortical adenoma, bilateral cortisol-secreting adenomas, or bilateral ACTH-independent macronodular adrenal hyperplasia (AIMAH) masquerading as bilateral single adenomas.…”