Searchable abstracts of presentations at key conferences in endocrinology

ea0063gp105 | Adrenal and Neuroendocrine - Basic | ECE2019

Reappraisal of the human adrenal cortex and fate

Camponova Pamela , Duparc Celine , Roy Malanie , Lefebvre Herve , Thomas Michael

The zonation of the human adrenal cortex has long been established morphologically and histologically as three distinct layers of cells. The outer zona glomerulosa (ZG) comprises densely packed cells arranged in clusters that produce aldosterone; the zona fasciculata (ZF) is composed of cells with large cytoplasm, containing lipid droplets arranged in radial columns that synthetize cortisol; and the zona reticularis is composed of compact and pigmented cells producing androgen...

ea0067o20 | Oral Presentations | EYES2019

Phenotype of patients carrying the c.709(-7-2)del PRKAR1A mutation in a large cohort of 40 patients

Abderrahmane Fatimetou , Raverot Gerald , Lefebvre Herve , Cardot-Bauters Catherine , Vantyghem Marie-Christine , Bertherat Jerome , Espiard Stephanie

Objective: To describe the Carney Complex (CNC) manifestations presented by patients harboring the PRKAR1A mutation c.709(-7-2)del (one of the three hotspots) in a large cohort of patients.Methods: Multicenter retrospective study. Age at the diagnosis or at the screening of the different CNC manifestations is described by mean ± standard deviation.Results: Forty patients [12 index cases, 27 females, 46±15 years o...

ea0022p51 | Adrenal | ECE2010

In vitro effect of serotonin (5-HT) on cortisol secretion in primary pigmented nodular adrenal disease

Louiset Estelle , Duparc Celine , Perraudin Veronique , Renouf Sylvie , Libe Rossella , Stratakis Constantine A , Bertherat Jerome , Lefebvre Herve

Illegitimate membrane receptors are known to play a role in cortisol secretion in adrenal adenomas and ACTH-independent macronodular adrenal hyperplasia (AIMAH) causing Cushing’s syndrome. Conversely, illegitimate receptors have never been described in primary pigmented nodular adrenal disease (PPNAD). In the normal adrenal gland, serotonin (5-HT) has been shown to stimulate cortisol secretion through activation of 5-HT receptor type 4 (5-HT4) whereas, in some AIMAH tissu...

ea0020s23.2 | Adrenocortical tumours – pathogenesis and management | ECE2009

Aberrant receptors (AR) in adrenal Cushing's syndrome

Libe Rossella , Groussin Lionel , Assie Guillaume , Bertagna Xavier , Chabre Olivier , Lefebvre Herve , Bertherat Jerome

Some elegant clinical observations of dysregulated cortisol-secretion in cases of ACTH-independent macronodular hyperplasia (AIMAH) and more rarely adrenocortical adenomas (ACA) have led to the concept of ‘aberrant’ or ‘illegitimate’ membrane receptors (AR) in adrenal Cushing syndrome. It this situation cortisol secretion is regulated by an extra-cellular ligand that usually does not stimulate cortisol secretion in normal adrenals. This abnormal response mi...

ea0016p60 | Adrenal | ECE2008

Intraadrenal production of ACTH in macronodular bilateral adrenal hyperplasia causing Cushing's syndrome: its role in the physiopathology of hypercortisolism

Louiset Estelle , Duparc Celine , Groussin Lionel , Bertherat Jerome , Bertagna Xavier , Kuhn Jean-Marc , Lefebvre Herve

Illicit expression of membrane receptors for circulating regulatory factors, such as GIP and LH receptors, has been well documented in ACTH-independent macronodular adrenal hyperplasias (AIMAHs) causing Cushing’s syndrome. In addition, we have observed an abnormal expression of serotonin, arginine vasopressin and ACTH in a subpopulation of steroidogenic cells in two AIMAH tissues. The aim of the present study was: (i) to investigate the presence of ACTH by immunohistochem...

ea0026p16 | Adrenal cortex | ECE2011

Potential role of mast cells in the physiopathology of aldosterone-producing adenoma

Duparc Celine , Moreau Lucile , Perraudin Veronique , Gobet Francoise , Meatchi Tchao , Plouin Pierre-Francois , Zennaro Maria-Christina , Louiset Estelle , Lefebvre Herve

We have shown that 5-hydroxytryptamine (5-HT) receptor type 4 (5-HT4R) agonists stimulate aldosterone production in patients with aldosterone-producing adenoma (APA). Moreover, 5-HT-positive cells were observed in APA tissues and 5-HT4R mRNAs were markedly overexpressed in APAs in comparison with normal adrenals. All these results suggested that 5-HT produced by adrenal mast cells could be involved in the physiopathology of APAs. The aim of this work was to investigate the rol...

ea0016p61 | Adrenal | ECE2008

A case of renin-producing adrenocortical carcinoma with Cushing's syndrome: in vitro effect of serotonin on renin and cortisol secretions

Louiset Estelle , Isvi Karen , Gasc Jean-Marie , Duparc Celine , Cauliez Bruno , Laquerriere Annie , Kuhn Jean-Marc , Lefebvre Herve

A 48-year-old woman was referred for ACTH-independent Cushing’s syndrome associated with a left adrenal tumor. Contrasting with hypercortisolism-related hypertension and hypokalemia, renin and aldosterone levels were paradoxically increased. The tumor was surgically removed and pathological examination of the tissue established the diagnosis of corticosurrenaloma. Fragments of the tumor were obtained for in situ hybridization, immunohistochemical and cell incubatio...

ea0063p814 | Adrenal and Neuroendocrine Tumours 3 | ECE2019

Abnormal serotonin regulatory loop in adrenals of patients with Cushing’s syndrome and 21-hydroxylase deficiency

Le Mestre Julie , Duparc Celine , Reznik Yves , Bonnet-Serrano Fideline , Touraine Philippe , Chabre Olivier , Young Jacques , Sibony Mathilde , Gobet Francoise , Raverot Gerald , Bertherat Jerome , Louiset Estelle , Lefebvre Herve

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...

ea0063p826 | Adrenal and Neuroendocrine Tumours 3 | ECE2019

The French COMETE-Cancer network for adrenal cancer: 10 years of activity as part of a national plan for clinical care of rare cancers

Libe Rossella , Tabarin Antoine , Chabre Olivier , Laboureau Sandrine , Goichot Bernard , Vezzosi Delphine , Lefebvre Herve , Verges Bruno , Niccoli Patricia , Vanthyghem Marie-Christine , Baudin Eric , Bertherat Jerome

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....

ea0049gp12 | Adrenal 2 | ECE2017

Activation of the cAMP/PKA transduction system triggers abnormal expression of the serotonin signaling pathway in human adrenocortical cells

Mestre Julie Le , Duparc Celine , Bram Zakariae , Reznik Yves , Bertherat Jerome , Touraine Philippe , Young Jacques , Chabre Olivier , Stratakis Constantine A. , Lefebvre Herve , Louiset Estelle

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...