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The article presents a case study of a 27-year-old woman with biochemical evidence of recurrence of disease after bilateral total adrenalectomy. Topics include pheochromocytomas being neuroendocrine tumors with highly variable clinical presentation; and functional imaging being used to confirm suspicious lesions found through CT or MRI after biochemical evidence of a pheochromocytoma.

Background: Various diagnostic tests are available to establish the primary aldosteronism (PA) diagnosis and to determine the disease laterality. Combined with the controversies in the literature, unawareness of guidelines and technical demands and high costs of some of these diagnostics, this could lead to significant differences in work-up strategies worldwide. Therefore, we investigated the work-up before surgery for PA in daily clinical practice within a multicenter study. Methods: Patients who underwent unilateral adrenalectomy for PA within 16 centers in Europe, Canada, Australia and the USA between 2010 and 2016 were included. We did not exclude patients based on the performed diagnostic tests during work-up to make our data representative for current clinical practice. Adherence to the Endocrine Society Guideline and variables associated with not performing adrenal venous sampling (AVS) were analyzed. Results: In total, 435 patients were eligible. An aldosterone-to-renin ratio, confirmatory test, computed tomography (CT), magnetic resonance imaging and AVS were performed in 82.9%, 32.9%, 86.9%, 17.0% and 65.3% of patients, respectively. A complete work-up, as recommended by the guideline, was performed in 13.1% of patients. Bilateral disease or normal adrenal anatomy on CT (OR 16.19; CI 3.50–74.99), smaller tumor size on CT (OR 0.06; CI 0.04–0.08) and presence of hypokalemia (OR 2.00; CI 1.19–3.32) were independently associated with performing AVS. Conclusions: This study is the first to examine the daily clinical practice work-up of PA within a worldwide cohort of surgical patients. The results demonstrate significant variability in work-up strategies and low adherence to The Endocrine Society guideline. [ABSTRACT FROM AUTHOR]