Endocrine Abstracts

Adrenal incidentalomas (AI) are recognized more and more frequently, due to the widespread use of CT. Although unexpected, these tumors raise the question of a putative morbidity related either to their proliferation potential or to their endocrine secretion. Regarding proliferation it is now established that a low (<10 UH) and homogeneous density on unenhanced CT scan is specific for a benign adrenocortical adenoma, which do not need to be operated for fear that they may ...

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...

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...

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...

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...

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...

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 ...

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...

Adrenocortical carcinoma (ACC) is a rare malignancy with dismal prognosis. Deregulated microRNA (miRNA) expression has been implicated in ACC aggressiveness. Nevertheless, the mechanisms underlying such deregulations remain unknown. Aberrant Wnt/β-Catenin signaling has been reported in about 40% of ACC and is associated with poor outcome. In the present work, we aimed to investigate the link between constitutive activation of Wnt/β-Catenin pathway and miRNA expressio...

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...