Searchable abstracts of presentations at key conferences in endocrinology

ea0081p678 | Pituitary and Neuroendocrinology | ECE2022

Treatment of cushing’s disease (CD) after primary failure of pituitary surgery or recurrence: evaluation of long-term control by medical treatment

Ghalawinji Adel , Cristante Justine , Lefournier Virginie , Chaffanjon Philippe , muller Marie , Gay Emmanuel , Olivier Chabre

Introduction: Pituitary surgery is the first line treatment for most patients with Cushing’s disease (CD) but after failure or recurrence after surgery 3 main strategies can be proposed: medical treatment (MT), a 2 nd pituitary surgery, bilateral adrenalectomy (BA). Pituitary radiotherapy is a 4th strategy, generally combined with one of the 3 others. Medical treatment (MT) emerges as an attractive strategy but there are little data on long-term control after f...

ea0063oc10.2 | Adrenal 2 | ECE2019

Glucocorticoid resistance patients exhibit defective cortisol metabolism, responsible for functional hypermineralocorticism

Vitellius Geraldine , Delemer Brigitte , Chabre Olivier , Caron Philippe , Pussard Eric , Bouligand Jerome , Trabado Severine , Lombes Marc

Glucocorticoid resistance syndrome, a rare genetic disease, is often associated with glucocorticoid receptor (GR) loss-of-function mutations. Six patients carrying heterozygous mutations of NR3C1 gene encoding GR, either missense R477S, Q501H, L672P or non-sense R469X, R491X, Y660X mutations were studied. Surprisingly, NR3C1 mutation carriers presented with low kalemia, low plasma renin and aldosterone levels associated or not with arterial hypertension, cons...

ea0063gp191 | Adrenal and Neuroendocrine - Clinical | ECE2019

Pneumocystis pneumonia as a major complication of medical treatment for severe Cushing’s syndrome linked either to ectopic ACTH secretion or to Cushing’s disease

Cristante Justine , Mallaret Michel , Tourvieilhe Laura , Carreau Agnes , Vaillant Tiphaine , Yoldjian Isabelle , Chabre Olivier

Several cases of pneumocystosis developing after lowering cortisol serum level have been reported, mostly in patients with ectopic Cushing’s syndrome. We report here 2 cases of pneumocystosis which developed after initiation of treatment with metyrapone, one in a patient with Cushing’s disease, and the second in a patient with ectopic ACTH secretion by a prostate endocrine carcinoma. Both cases presented severe Cushing’s syndrome. We then analyzed the periodic s...

ea0041ep27 | Adrenal cortex (to include Cushing's) | ECE2016

Bilateral adrenalectomy in Cushing’s syndrome: evaluation of quality of life compared to other treatment options

Sarkis Pauline , Rabilloud Muriel , Siamand Anna , Lifante Jean-Christophe , Chabre Olivier , Raverot Gerald

Context: Bilateral adrenalectomy (BADX) has become an important treatment option of Cushing’s syndrome (CS), especially when other treatments have failed. Long-term outcome is not well known.Objective: To evaluate long-term quality of life (QoL) of patients who underwent BADX for CS compared to other therapeutic options.Methods: Fourty five patients with all cause CS were identified: 23 patients had BADX and 22 underwent one o...

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...

ea0020p662 | Reproduction | ECE2009

Ovulation induction and multicentric follow-up of 21 pregnancies in 14 patients

Villaret Laure , Chabre Olivier , Caron Philippe , Young Jacques , Quenard Nicole , Bry Helene , Hoffmann Pascale

Objective: To determine the outcome of pregnancy in hypopituitary women.Background: Rare situation, not studied enough: only two previous studies concerning 9 and 19 patients (Overton et Hall).Design: Multicentric, descriptive, prospective and retrospective study.Material and methods: Twenty-one pregnancies were registered in 14 hypopituitary women (defined as gonadotropic deficiency associated with at least ...

ea0056p837 | Pituitary - Clinical | ECE2018

Cushing’s disease with negative or inconclusive MRI: reassessment of transphenoidal surgery at the age of medical treatment. Post operative remission rate in 184 patients including 86 with negative or inconclusive MRI

Cristante Justine , Lefournier Virginie , Sturm Nathalie , Guy Passagia Jean , Gay Emmanuel , Chabre Olivier

When pituitary MRI show a typical imaging of adenoma, it is agreed that transsphenoidal surgery is the reference treatment with remission achieved in about 80% of patients. If MRI is negative or inconclusive, some authors consider that the results of surgery are less successful, and propose medical treatment as a first line therapy, despite a disease control rate between 30 and 70%. Since 1990, our center chose to systematically explore patients with ACTH dependent hypercortic...

ea0014oc3.2 | Endocrine tumors & neoplasia | ECE2007

Adrenal lesions in multiple endocrine neoplasia type 1: data from the French Group for the Study of Endocrine Tumors (GTE)

Gatta Blandine , Monsaingeon Maud , Goudet Pierre , Murat Arnaud , Niccoli-Sire Patricia , Calender Alain , Rohmer Vincent , Chabre Olivier , Tabarin Antoine

The characteristics of adrenal involvement in Multiple Endocrine Neoplasia type 1 (MEN1) have been defined from studies involving a limited number of patients. We have assessed retrospectively the prevalence, characteristics and evolution of adrenal involvement from the French group for the study of endocrine tumours (GTE) registry, involving 688 patients with MEN1. In our series, adrenal tumours identified at abdominal imaging occurred in 130 patients (18.9%). The mean age of...

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....