Endocrine Abstracts

Introduction: Aggressive ACTH-independent Cushing’s syndrome is rare and frequently related to malignant adrenocortical tumor. However, in exceptional situations, ectopic steroids secretion has been described.Case report: We report the case of an 87 years-old woman referred to our clinic in September 2020 after a low trauma sacral fracture. Her recent medical history revealed the onset, in the previous months, of severe hypokalemia, uncontrolled typ...

Introduction Primary bilateral macronodular adrenal hyperplasia (PBMAH) is potentially responsible for variable degree of cortisol excess. In patients with PBMAH the complete remission of cortisol hypersecretion can be achieved only by performing bilateral adrenalectomy, leading to a persistent hypocortisolism and to a consequent need of a lifelong glucocorticoid replacement therapy. Therefore, bilateral adrenalectomy is worth doing only in patients with severe hypercortisolis...

Introduction: Primary aldosteronism (PA) has been described in association with endocrine and non-endocrine neoplasms. Aldosterone-producing adenomas mainly associate with hyperparathyroidism, prolactinomas and pancreatic endocrine tumors, particularly in the context of multiple endocrine neoplasia type 1 MEN1 syndrome. Next-generation sequencing (NGS) studies have shown frequent somatic mutations underlying PA and, rarely, germline mutations of CYP11B1/CYP11B2</e...

Background: The Endocrine Society Guidelines recommend screening for hypercortisolism especially in patients with specific features that best discriminate Cushing’s Syndrome (CS): easy bruising, facial plethora, proximal myopathy and striae. Clinical experience suggests that these features, though suggestive of hypercortisolism, are not enough sensitive. Indeed, patients with hypercortisolism frequently manifest primarily less discriminatory cortisol-related features, suc...

Primary pigmented nodular adrenocortical disease (PPNAD) is a rare form of ACTH-independent Cushing’s syndrome (CS). Approximately 30% of patients with PPNAD are sporadic cases and the other are familial. We describe a case of a patient affected by PPNAD presenting with isolated arterial hypertension. A 19-year-old black female patient was referred to the Endocrinology Unit in september-2021 due to severe arterial hypertension and fatigue. Previous clinical history was un...

Introduction: The management of patients with adrenal incidentaloma (AI) and possible subclinical hypercortisolism (SH) is debated. This randomized study was aimed to evaluate the effects of adrenalectomy on arterial hypertension (AH).Methods: We consecutively evaluated 590 AI patients (referred to 3 Italian Centres between 06/2016 and 12/2019). Among these, 134 patients showed a possible SH (i.e. 1 mg DST between 1.8 and 5 µg/dl). Based on the excl...

IntroductionThe management of patients with adrenal incidentaloma (AI) and mild autonomous cortisol secretion (MACS) is debated. This randomized study aimed to evaluate the effects of adrenalectomy on arterial hypertension (AH), glucose and lipid metabolism.MethodsWe consecutively evaluated 626 AI patients (referred to 3 Italian Centers between 06/2016 and 02/2020). According to the inclusion criteria, we enr...

IntroductionIrisin is a molecule secreted from skeletal muscle and contributes to maintenance of metabolic homeostasis leading to increased energy expenditure and reducing the risk of obesity and diabetes. Literature data suggest that patients with Cushing disease have lower irisin values than controls and remission of hypercortisolism increases these values. Moreover, other plasma inflammation markers implicated in the pathogenesis of atherosclerosis, i...

Most adrenal incidentalomas are benign and can be divided into nonfunctioning adrenal tumors (NFAT) and tumors with mild autonomous cortisol secretion (MACS). Several studies suggest that MACS may result in an increased risk for mortality and cardiometabolic disease. The cardiometabolic risk in MACS is possibly related to the increased frequency of cardiovascular risk factors such as diabetes mellitus (DM) and hypertension (HT) induced by cortisol excess. This is confirmed by ...

Cushing syndrome (CS) is a chronic and debilitating condition with high morbidity and mortality. Development of novel, safe, and effective pharmacologic therapies with improved risk-benefit profiles would enrich treatment options for patients. Relacorilant is a selective glucocorticoid receptor (GR) modulator that competitively antagonizes cortisol activity and, unlike the FDA-approved GR antagonist mifepristone, does not bind to the progesterone receptor. In a phase 2 study i...