Endocrine Abstracts

Context: Mosaicism is a feature of several inherited tumor syndromes but is rarely systematically looked for in routine. MEN1 is an autosomal dominant hereditary syndrome characterized by several endocrine tumors affecting parathyroids, pancreas, and anterior pituitary most of the time, due to inactivating mutations in the MEN1 gene. Few cases of mosaicism in Multiple Endocrine Neoplasia type 1 (MEN1) have been described. MEN1 mosaicism is probably under-diag...

Background: Medical treatment with cortisol-lowering drugs is commonly used following pituitary surgical failure or recurrence of hypercortisolism in patients with Cushing’s disease (CD). Studies using late-night salivary cortisol (LNSF) measurement have shown persistent disruption of the circadian secretion of cortisol despite normalization of UFC in a subset of medically treated CD patients. Study aim Our objective was to assess the long-term cortisol exposure in CD pat...

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....

Introduction: Acromegaly is characterized by excessive secretion of GH and increased IGF-1 levels caused by benign pituitary adenoma. The ACRO-POLIS study describes symptoms and comorbidities of acromegaly at diagnosis in a large cohort of patients diagnosed between 2009 and 2014 in France.Methodology: Observational, cross-sectional, multicentre study included adult patients with acromegaly diagnosed for less than 5 years. Data were collected retrospecti...

Introduction: Acromegaly is characterised by chronic, excessive secretion of GH and increased IGF1 levels caused by benign pituitary adenoma. This study aimed at describing the symptoms and comorbidities of acromegaly at diagnosis, in a large cohort of patients diagnosed between 2009 and 2014.Methods: Observational, cross-sectional, multicentre study conducted in France between September 2013 and June 2014. Adult patients with acromegaly diagnosed for &#...

The loss of nychthemeral rhythm is a feature of endogenous hypercorticism that may be investigated by the assay of midnight salivary cortisol (serC0h) although often replaced by serum cortisol assay. However, manufacturers of cortisol immunoassays kits seldom provide reference values for this horary. We present here tentative reference values of serC0h with an automated imunoanalyzer (DXi800, Beckman Coulter).The population retrospe...

Chronic exposure to hypercortisolism significantly impacts on patient’s health and health-related quality of life (HRQoL). We developed a disease-generated questio-nnaire to evaluate HRQoL in Cushing’s syndrome (CS) (CushingQoL); in 125 (104 females) patients recruited in Spain, France, Germany, The Netherlands and Italy, clinical and hormonal data were collected and correlated with results of the generic SF-36 questionnaire, a question on self-perceived general heal...

Introduction: LNSC has shown high sensitivity and specificity for the initial diagnosis of CD and detection of disease recurrence; however, the use of LNSC to monitor medical treatment of CD is not well established. The results of an exploratory analysis evaluating changes in LNSC in CD patients receiving long-acting pasireotide during a Phase III study (CSOM230G2304; Lacroix et al. Lancet Diabetes Endocrinol 2018) are reported here.Methods: Pat...

Acromegaly is a morbid condition mainly caused by overproduction of growth-hormone (GH) from a pituitary adenoma leading to excessive growth. Normalisation of insulin-like growth factor-1 (IGF1) is an important goal for the treatment of acromegaly. The second-generation somatostatin analog (SSA) long acting pasireotide (la-PAS) has recently been introduced for the management of patients uncontrolled by first-generation SSA. The ACRONIS study (CSOM230CIC05) will provide real-wo...

Introduction: Potent 11β-hydroxylase inhibitor osilodrostat provides cortisol level control in patients with Cushing’s syndrome (CS), as demonstrated by the LINC clinical development programme in Cushing’s disease (CD) patients.1 We report data from year 1 of the prospective observational LINC6 study (NCT05382156), evaluating long-term safety and efficacy of osilodrostat in CS patients during 3 years of routine clinical practice.<p class="abstext"...