Cushing's Syndrome by Left Adrenocortical Adenoma Synchronously Associated with Primary Aldosteronism by Right Adrenocortical Adenoma: Report of a Case

Abstract

mal range; 30-159) pg/ml and 10 (4.4-17.4) µg/dl, respectively. Although her plasma renin activity (PRA) and ACTH was suppressed to an undetectable level, no further examination was conducted or followedup. One year later, hypertension and progressive edema in the lower extremities gradually appeared. Reexamination revealed hypopotassemia and elevation of plasma aldosterone level as high as 216 (normal range; 30-159) pg/ml, with low PRA as 0.3 (0.5-2.0) ng/ml/ hr. Magnetic resonance imaging demonstrated that the right adrenal mass did not enlarge. Abstract. Synchronous associations of Cushing's syndrome (CS) and primary aldosteronism (PA) with multiple adrenocortical adenomas secreting each hormone independently have rarely been reported. Herein, we describe a unique case of PA associated with CS with detailed clinical and pathological investigations. bilateral adrenal masses with clinical symptoms of CS and PA were found in a 43-year-old woman. Venous sampling demonstrated excess secretion of cortisol, and aldosterone from right, and left tumor, respectively. A bilateral laparoscopic partial adrenalectomy was undergone. The right adrenal tumor (3 cm) was yellow in color with abundant lipofuscin granules, and was composed of both eosinophilic compact cells and clear cells. In situ hybridization showed that both mRNAs for HSd3b2 and CYP17A1 were strongly expressed in the tumor, suggesting cortisol synthesis. Left adrenal tumor (2.4 cm) was golden-yellow in color, and composed of clear cells only. expression of HSd3b2 and CYP11b mRNAs were observed in the tumor compatible with the aldosterone synthesis. Furthermore, minute nodules were found at the surface of normal-appearing cortex on both sides of the adrenal glands, and the expression of HSd3b2 and CYP11b mRNAs was clearly demonstrated within the nodules, indicating aldosterone synthesis. we diagnosed that the present case had 1) cortisol-producing right adrenocortical adenoma, 2) aldosterone producing left adrenocortical adenoma, and 3) cortical minute nodules with aldosterone production in both adrenal glands compatible with idiopathic adrenal hyperplasia. we reviewed the cases reported, and discussed the significance of the minute nodules in the adrenal cortex, often found in association with the adrenocortical adenoma.Key words: Adrenal gland, Cushing's syndrome, primary aldosteronism, bilateral adrenocortical adenomas, Laparoscopic adrenalectomy (Endocrine Journal 56: [495][496][497][498][499][500][501][502] 2009) A JApAnese woman was admitted to a local hospital for the treatment of her depression when she was age 35. Right adrenal mass of less than 20 mm was demonstrated by imaging studies during her workup. At this time, both plasma aldosterone and plasma cortisol levels were within normal limits, at 72 (nor-

cAMP/PKA signaling defects in tumors: Genetics and tissue-specific pluripotential cell-derived lesions in human and mouse

“…BAHs are mostly cortisol- or aldosterone-producing (Stratakis and Boikos, 2007; Stratakis; 2008; Gunther et al, 2004; Bourdeau et al, 2007; Geller et al, 2008), lately, an adrenal androgen-producing form of BAH was also described (Ghayee et al, 2011). Combined gluco- and mineralo-corticoid hormone-producing adrenal lesions have also been described (Onoda et al, 2009; Murakoshi et al, 1995), and we have seen them in our patients; almost certainly BAHs that produce sex steroids in combination with gluco- or mineralo-corticoid hormones also exist (unpublished data). On the basis of size, cortisol-producing BAHs are divided into two groups: macronodular (when the size of their nodules mostly exceed 1 cm) and micronodular (when nodules are smaller than 1 cm) (Stratakis and Boikos, 2007; Stratakis; 2008; Bourdeau et al, 2007).…”

Difficult-to-control hypertension due to bilateral aldosterone-producing adrenocortical microadenomas associated with a cortisol-producing adrenal macroadenoma

“…Although several cases of simultaneous hypersecretion of cortisol and aldosterone have been reported, most of them were classified into two groups: one because of a single, sometimes multiple, adrenocortical macroadenoma(s) that produced both cortisol and aldosterone, 9, 10, 11, 12, 13, 14 and the other because of a combination of cortisol-producing macroadenoma(s) and aldosterone-producing macroadenoma(s). 15, 16, 17, 18 Furthermore, the preoperative diagnosis of most of these cases were made on radiological findings obtained by conventional CT as well as on endocrinological findings. 9, 11, 12, 15, 16 In this case, we preoperatively suspected the hypersecretion of aldosterone from bilateral adrenals from the results of AVS, and confirmed bilateral microAPAs as the cause of aldosterone excess by immunohistochemical analysis of steroidogenic enzymes.…”

Bilateral Adrenocortical Masses Producing Aldosterone and Cortisol Independently

“…However, cases of multiple simultaneous adrenocortical adenomas secreting each hormone independently are extremely rare, particularly when those adenomas are located in different adrenal glands. As far as we can find from the database of PubMed and KoreaMed, only four cases have been reported in English, Japanese, and Korean [ 16 17 18 19 ]. The first such case in Korea was reported by Choi et al [ 16 ], who described a patient having primary hyperaldosteronism and Cushing syndrome with bilateral adrenal masses.…”