Endocrine Abstracts

In the human adrenal gland, serotonin (5-HT), released by subcapsular mast cells stimulates corticosteroid secretion through activation of type 4 serotonin receptors (5-HT4R) positively coupled to cAMP/proteine kinase A (PKA) signaling pathway and calcium influx. The 5-HT4R is principally expressed in zona glomerulosa cells explaining why 5-HT strongly stimulates aldosterone production but only exerts a modest stimulatory action on cortisol. Interestingly, in primary pigmented...

Introduction: The French National Institute of Cancer (INCa) launched supported by the Ministry of Health in 2008 a program for the recognition of national networks for the management of rare cancers. Among the 23 selected networks COMETE-Cancer was recognized by INCa in 2009 for Adrenocortical carcinoma (ACC) and malignant pheochromocytoma/paraganglioma (MPP). At that time 60–120 new ACC/year and 30 new MPP/year were expected at the national level....

In human adrenals, serotonin (5-HT), released by subcapsular mast cells, increases aldosterone secretion through activation of type 4 serotonin receptors (5-HT4R) but only exerts a modest control on cortisol production. Interestingly, illicit synthesis of 5-HT in adrenocortical cells as well as overexpression of the 5-HT4R and ectopic expression of the type 7 receptor (5-HT7R) have been observed in bilateral macronodular adrenal hyperplasia res...

Background: Pregnancy in women with hypopituitarism has been described as presenting high risks of maternal and fetal complications but this is only based on 2 small size series.Objective: To reassess the prognosis of pregnancy in women with hypopituitarism.Methods: Multicentric, observational, retrospective study including 39 pregnancies in 28 women with gonadotropic insufficiency and at least one other pituitary hormone deficienc...

Introduction: In patients with primary aldosteronism (PA) international consensus claim that adrenal venous sampling (AVS) performs better than CT scan in determining lateralization of aldosterone secretion. The SPARTACUS study has however reported similar performances for lateralization by CT scan and AVS in patients with PA and a unilateral adrenal mass. The standard procedure for AVS uses cortisol values to validate the selectivity of catheterization and to normalize the al...

For all ERCUSYN investigatorsBackground: Patients with Cushing’s syndrome (CS) have an increased risk of developing venous thromboembolic complications. There is currently no standard practise for thromboprophylaxis in CS patients.Aim: To study the details of VTE in patients included in The European Registry on Cushing’s syndrome (ERCUSYN) and study various thromboprophylaxis protocols used among the centres where VTE&#14...

Background: Hyperphagia leading to craniopharyngioma-related obesity (CRO) is a common and serious sequel of treatments of craniopharyngiomas. The few therapeutic approaches that have been tested until now for the control of eating behaviour and weight have poor efficacy. Glucagon-like peptide-1 (GLP-1) analogues might be an option. Methods: This multicentre, randomised, double-blind superiority trial was conducted in France. Adults with CRO (BMI > 3...

Introduction: The Carney Complex is a multiple endocrine and non endocrine neoplasia mostly due to PRKAR1A mutations. Spectrum of manifestations and genotype-phenotype correlations have been previously described by retrospective studies. A prospective study evaluating the occurrence of the different manifestations was needed to precise the optimum follow-up.Methods: Multi-center national prospective study (Clinical Trials NCT00668291) including 70 patien...

The European Registry on Cushing’s syndrome (ERCUSYN) is designed to collect prospective and follow-up data at EU level on patients with Cushing’s syndrome (CS). Baseline data on 481 CS patients (390 females, 91 males; mean age (±S.D.): 44±14 years) collected from 36 centres in 23 countries. This cohort included new patients since 2008 and retrospective cases since 2005. Patients were divided into four major etiologic groups: pituitary-depend...

Background: Osilodrostat is a potent oral inhibitor of the adrenal enzymes aldosterone synthase and 11b-hydroxylase and decreases glucocorticoid and mineralocorticoid production and secretion. Phase 2 and 3 studies from the osilodrostat clinical trial programme have demonstrated the drug’s efficacy and safety in patients with Cushing’s disease. Osilodrostat received European Marketing Authorization (MA) for the treatment of Cushing’s syndrome (CS) in adults....