Endocrine Abstracts

Cushing’s syndrome can occur as a consequence of prolonged exposure to excessive amounts of circulating free cortisol and the various causes can be broken down into exogenous causes, such as from steroid treatment for chronic illnesses, or endogenous as in this case. Cushing’s syndrome can be further classified into ACTH-dependent and ACTH-independent1, for which the latter accounts for 15–20% of all cases. This report highlights the case of a 35 year old female...

Purpose: Adrenal vein sampling (AVS) is the gold standard method for subtyping primary aldosteronism (PA). A certain number of PA patients had unilateral successful AVS, concomitant autonomous cortisol secretion or indeterminate lateralization index (LI 2.5–4), which made correct interpretation of AVS data impossible. The aim of our study was to determine whether absolute aldosterone concentration can predict the subtype of PA in patients with inconclusive AVS data.<p...

Background: One in 5 million children is affected by adrenocortical carcinoma (ACC) each year. Since prognosis for children older than 4 years is limited, clinicians often choose aggressive treatment with etoposide, doxorubicin, cisplatin (EDP) and mitotane after resection. However, little is known about the impact of EDP-mitotane on development in children. We therefore aimed to provide an overview of side-effects and neurocognitive effects of EDP-mitotane in children.<p ...

Background: Ectopic Cushing’s syndrome (ECS) frequently represents a diagnostic challenge due to its complex clinical presentation.Case presentation: A 57-year-old male presented with typical Cushingoid appearance, high adrenocorticotropic hormone (ACTH) level (138.3 pg/ml), lack of cortisol circadian rhythm and suppression in overnight 1 mg dexamethasone suppression test (DST) (1374 nmol/l). Great daily fluctuation of serum cortisol levels from ver...

Introduction: Measurement of the baseline level of 17-hydroxyprogesterone (17OHP) is a screening test for NCAH, a less severe form of congenital adrenal hyperplasia.Aim: A real-life verification of the currently adopted 17OHP threshold (≥2.0 ng/ml) at which the further diagnostic (cosyntropin stimulation test) should be performed.Material and methods: The study included 400 patients (385 females and 15 males) referred to the ...

Background: Hirsutism is a common and disturbing complaint among women attending endocrine clinics. It is caused by androgen excess and when severe, might progress to virilization. Virilizing adrenal tumors are a rare cause of hirsutism.Case presentation: A 14 year old girl presented with primary amenorrhea, poor breast development (Tanner stage B1), hirsutism (Ferriman-gallwey score, FGS of 12/36) clitoromegaly (clitoral index >200 mm2). ...

Background: Diffuse large B-cell lymphoma is the most common non-Hodgkin’s lymphoma (NHL). It can involve extranodal sites in about 30%, but the involvement of endocrine organs is rare (adrenal involvement is estimated<0.2%) and correlates with poor prognosis. In contrast to pituitary metastases from solid tumors, NHL involvement of the anterior lobe of the pituitary is more frequently seen.Case presentation: A 84-year-old woman presented to the...

Background: Ectopic ACTH secretion from metastatic acinic cell carcinoma (ACC) of the parotid gland is extremely rare. ACC is an uncommon, typically indolent, salivary gland neoplasm. Herein, we report a case of a high-grade ACC later diagnosed with Cushing’s syndrome (CS).Case presentation: A 60-year-old man presented with a laterocervical mass. Computed tomography (CT) documented a 39×38 mm mass on the parotid gland and homolateral cervical l...

Introduction: Retrospective analyses suggest an increased risk for cardiovascular diseases in patients with adrenal insufficiency (AI), mainly due to supraphysiological replacement doses.Objective: To evaluate the 24-hour blood pressure (BP) profile in patients with primary (PAI) and secondary (SAI) AI.Material and methods: BP threshold criteria for hypertension and dipping status of the 2018 ESC/ESH guidelines were used (24-h: &#8...

Background: Patients with adrenal incidentalomas and possible autonomous cortisol secretion (pACS) suffer from a high rate of cardiovascular events and mortality. We have previously showed that left ventricular mass index (LVMi) is higher in patients with pACS, compared with patients with non-functioning adenomas (NFA). To date, the management of pACS is still controversial, with a lack of consensus regarding the benefits of surgery.Objectives: This pros...

Background: The increase use of abdominal imaging have given rise in incidental discovery of adrenal masses. We report a case of a localized Castleman’s Disease(CD), mimicking an adrenal mass.Case report: A 29-year-old woman was found to have a 6 cm, well-circumscribed and hypoechoic, right adrenal incidentaloma during an abdominopelvic ultrasonography. The CT scan confirmed a suprarenal mass with heterogenous contrast enhancement. Adrenal gland cou...

Background: Cushing’s syndrome (CS) is characterized by inappropriate cortisol secretion and its diagnosis is still challenging for endocrinologist. Nevertheless, CS is a rare condition, making difficult to collect data and establish tests’ diagnostic performances.Objectives: Assessing diagnostic performances of Urinary Free Cortisol (UFC) evaluated by liquid chromatography method coupled with tandem mass (LC-MS/MS) and Late Night Salivary Cort...

Background: Mitotane is the only drug approved for the treatment of adrenocortical carcinoma (ACC). Although adverse effects (AEs) associated to its use are frequent, detailed information are very limited. Aim was to evaluate the AEs of mitotane monotherapy in ACC patients. Methods: We performed a retrospective multicenter study including 311 ACC patients (F=200, median age 49 yrs) treated with mitotane as first line of medical treatment. Presence and grade of AEs were collect...

Background: Adrenal insufficiency (AI) is a rare condition affecting children, potentially life-threatening. Autoimmune adrenalitis accounts for 15% of all cases of primary adrenal insufficiency in pediatric age. Most symptoms are usually unspecific, making the diagnosis quite challenging.Objective: The aim of the study was to describe clinical presentation, biochemical abnormalities, treatment and the clinical course of patients with autoimmune AI.<...

Background: Mitotane has been used in paliative setting in patients with adrenocortical carcinoma. However, this adrenolytic agent has important toxicities with frequent elevation of cholesterol levels (after a median time of 6 months from mitotane start) and serum aminotransferases. However, there are no published reports of clinically apparent liver injury.Case presentation: We report a case of a 54 year-old man with history of an 11 cm adrenocortical ...

Background: A pheochromocytoma is a rare neuroendocrine tumor, originating from adrenomedullary chromaffin cells. The pre-surgical management of pheochromocytoma is classically performed with alpha-blockers. However, in some cases other pharmacological approaches can be considered.Objective: The aim of the study is to describe the clinical course of patients with pheochromocytoma prepared for surgery with calcium channel blockers (CCB).<p class="abst...

Introduction: Transcriptional regulation of gene expression by miRNAs is critical for the fine-tuning of adrenal stress response. However, its role in hypercortisolism has not been explored well. The study addresses this gap using adrenal samples of 3 patient groups from the German Cushing’s registry: Cortisol-Producing-Adenoma (CPA), Primary Bilateral Adrenal Hyperplasia (PBMAH) and controls (adrenal samples of patients with pheochromocytoma).Metho...

Background: An increasing number of mutations are associated with pheochromocytomas. Genetic screening is advocated in all cases and immunohistochemistry as well as phenotype profile recognition may permit a more targeted screening for specific genes.Case Presentation: A 54-year-old male presented with symptoms compatible with pheochromocytoma and increased levels of plasma normetanephrines, metanephrines and 3-methoxytyramine. On Computed Tomography, th...

Background: In patients with adrenal nodules increased 17-OH Progesterone (17-OHP) does not necessarily indicate congenital adrenal hyperplasia (CAH) since adrenocortical tumors may harbor defects in steroidogenesis resulting in high steroid precursors, either basally or, usually, after Synacthen stimulation. Secretion of precursors has been associated with malignancy but it can also be present in benign adenomas.Case Presentation: A 62-year-old lady wit...

Background: ACTH-dependent Cushing’s syndrome (CS) is mostly associated with corticotroph adenomas or, infrequently, ectopic ACTH secretion(EAS). Adrenals may show diffuse bilateral enlargement or even macronodules. Adrenal metastases may occur in various cancers. They are often bilateral, irregular, with attenuation values >20 Hounsfield units(HU) on unenhanced CT scan and elevated SUVmax on FDG-PET scan. They may cause adrenal insufficiency due to the destruction of...

Background: Patients with primary adrenal insufficiency (PAI) carry an increased risk of infections which can precipitate adrenal crises. They are thought to be more likely to contract COVID-19 and develop severe disease; however, little is known about the true impact of COVID-19 on these patients.Objectives: To assess the response of a large single-centre cohort of PAI patients to COVID-19, focusing on morbidity and health promotion attitudes.<p cla...

Background: Primary bilateral macronodular adrenocortical hyperplasia (PBMAH) is a rare cause of ACTH-independent Cushing’s syndrome. The clinical application of adrenal venous sampling (AVS) in diagnostic work-up for PBMAH is still controversially discussed. AVS might be useful in evaluating the predominant side and guiding the decision for unilateral adrenalectomy in PBMAH.Objectives: Our aim was to evaluate whether certain metabolites in AVS samp...