Analysis of clinical and pathological features of primary bilateral macronodular adrenocortical hyperplasia compared with unilateral cortisol-secreting adrenal adenoma

Abstract

Background: Primary bilateral macronodular adrenocortical hyperplasia (PBMAH) is a rare condition with untypical subclinical symptoms of Cushing's syndrome (CS). This study aimed to compare the clinical and pathological features of PBMAH with unilateral cortisol-secreting adrenal adenoma (UAA).Methods: We prospectively included 46 PBMAH patients and 205 UAA patients from January 2000 to February 2014. Cortisol levels and 24 hours urine free cortisol (UFC) were determined at baseline and during dexamethasone suppression test (DST) using the chemiluminescence method. Computed tomography (CT) examination of the adrenal glands was performed in all patients. For patients treated with adrenalectomy, hematoxylin, and eosin, staining was performed for pathological examination.Results: The proportion of patients with autonomous cortisol secretion was significantly higher in PBMAH patients (39.1%) than UAA patients (6.8%). The PBMAH patients showed significantly lower levels of basal cortisol, low dose dexamethasone suppressed cortisol, and high dose dexamethasone suppressed cortisol than the UAA patients (452.6±183.3 vs. 578.7±166.4 nmol/L, P=0.003; 394.5±298.9 vs. 549.2±217.7 nmol/L, P=0.002; 397.3±282.3 vs. 544.3±187.6 nmol/L, P=0.003). Similarly, the PBMAH patients had significantly lower levels of basal 24 hours UFC, low dose dexamethasone suppressed 24 hours UFC, and high dose dexamethasone suppressed 24 hours UFC than the UAA patients (1,144.4±1,048.1

Beyond symptomatology: A comparative analysis of unilateral and bilateral macronodular mild autonomous cortisol secretion

“…29 Furthermore, in a study conducted by Zhang | 103 and hypertension were identified at a higher rate in the PBMAH than UAA, despite the fact that the patients with unilateral adenoma contained more cases of overt CS. 6 On the other hand, Morelli et al 30 investigated patients with subclinical hypercortisolism in both bilateral and UAA groups and no significant differences in dyslipidemia or T2DM were observed. However, Morelli et al…”

Molecular tools for diagnosing diseases of the adrenal cortex

“…Hormonal characterization includes clinical symptoms of Cushing syndrome, often associated with autonomous cortisol secretion and presentation of ACTH-independent hypercortisolism. Because hypercortisolism usually develops slowly and radiologic imaging lacks specificity, PBMAH diagnosis is challenging and often delayed [26]. However, the improvement of diagnostic methods for adrenal incidentalomas increased the frequency of PBMAH diagnosis, which advanced the understanding of the clinical heterogeneity of this disease.…”

Cushing´s syndrome due to bilateral adrenal cortical disease: Bilateral macronodular adrenal cortical disease and bilateral micronodular adrenal cortical disease

“…Other authors reported suppressed ACTH levels only in 30% of the cases (48). The dexamethasone-CRH test is not useful for the differential diagnosis of ACTH dependence since it can cause false positives in a high percentage of bilateral adrenal lesions (~40% of the cases) (50).…”

“…Moreover, in patients with hypertension and/or hypokalemia, primary hyperaldosteronism should also be discarded. In addition, since these are bilateral lesions, adrenal insufficiency should be evaluated if there is suspicion of malignant or infiltrative disease, and of CAH (46,48).…”