C-peptide level concomitant with hypoglycemia gives better performances than insulin for the diagnosis of endogenous hyperinsulinism: a single-center study of 159 fasting trials.

Introduction: Diagnosis of endogenous hyperinsulinism relies on the occurrence of a hypoglycemia, concomitant with inadequate high insulin and C-peptide levels. However, diagnostic cutoffs are not consensual among the different learned societies. The objective of this work was to propose optimized cutoffs for these three parameters for the diagnosis of endogenous hyperinsulinism.

Pneumocystis pneumonia in patients with Cushing's syndrome: A French multicenter retrospective study.

Objective: Pneumocystis pneumonia (PcP) is an opportunistic infection occurring in immunocompromised patients. Cushing's syndrome (CS) impairs the immune system, and several authors have reported PcP in patients with CS. The present study aimed to characterize PcP occurring in a CS context and its management in French tertiary centers, in order to highlight the similarities in clinical presentation and treatment according to whether prophylaxis is implemented or not.

Redifferentiating Effect of Larotrectinib in -Rearranged Advanced Radioactive-Iodine Refractory Thyroid Cancer.

Metastatic thyroid cancers may dedifferentiate and become radioactive-iodine (RAI) resistant. A redifferentiating effect can be observed with inhibitors of the mitogen-activated protein kinase pathway in thyroid cancers with point mutation in oncogenes. This effect allows RAI reuptake that may lead to a therapeutic effect different from the antitumoral effect of the inhibitor.

F-fluorocholine PET/CT in MEN1 Patients with Primary Hyperparathyroidism.

Background: Primary hyperparathyroidism (HPT1) is the most frequent endocrinopathy in multiple endocrine neoplasia type 1 (MEN1). Its surgical management is challenging. We aimed to describe and compare the imaging findings of parathyroid ultrasound (US), sestaMIBI scintigraphy (sestaMIBI), and F-fluorocholine (FCH) PET/CT in a series of MEN1 patients with HPT1.

Amiodarone-induced thyrotoxicosis.

Amiodarone-induced thyrotoxicosis (AIT) are not uncommon endocrinopathies. Clinicians are sometimes faced with difficult diagnostic and therapeutic situations. The disease pathophysiology is partially understood, explaining the lack of predictive factors for occurrence.

Prolonged response to Lu-DOTATATE therapy of a bone marrow infiltration in a refractory thymic neuro endocrine tumor.

Thymic neuro endocrine tumor (tNET) are extremely rare malignancies with poor prognosis, requiring investigation of novel therapeutic approaches. Lu-DOTATATE is a successful systemic treatment modality in patients with metastatic gastroenteropancreatic but it role in tNET is not yet well established. Here we report a case of a 39-year-old man with refractory bone marrow infiltration of a tNET, treated by 4 cycles of peptide receptor radionuclide therapy (PRRT) with Lu DOTATATE.

False-Positive Results in 18F-Fluorocholine PET/CT for a Thymoma in Workup of a Hereditary Primary Hyperparathyroidism.

We present the case of a severe familial primary hyperparathyroidism related to a germline deletion in the HRPT2 (CDC73) gene. Morphological explorations revealed 2 potential hyperfunctioning parathyroid glands: a left cervical lesion on the neck ultrasound, and an ectopic mediastinal lesion on the parathyroid scintigraphy using Tc-methoxyisobutylisonitrile and on F-fluorocholine PET/CT. Surgery removal and histopathological examination determined that the mediastinal mass corresponded to a thymoma and the cervical lesion to a parathyroid adenoma.

Pituitary Lesion of Unknown Origin: Think Epithelioid Angiosarcoma.

We report the unusual etiology of a sellar aggressive mass, an epithelioid angiosarcoma, the diagnosis of which was made from positive epithelial and vascular markers.

DUSP5 and DUSP6, two ERK specific phosphatases, are markers of a higher MAPK signaling activation in BRAF mutated thyroid cancers.

Background: Molecular alterations of the MAPK pathway are frequently observed in papillary thyroid carcinomas (PTCs). It leads to a constitutive activation of the signalling pathway through an increase in MEK and ERK phosphorylation. ERK is negatively feedback-regulated by Dual Specificity Phosphatases (DUSPs), especially two ERK-specific DUSPs, DUSP5 (nuclear) and DUSP6 (cytosolic).

Long-Term Control of Hypercortisolism by Vandetanib in a Case of Medullary Thyroid Carcinoma with a Somatic RET Mutation.

Background: Medullary thyroid carcinomas (MTCs) complicated by ectopic Cushing's syndrome (CS) have a poor prognosis, partially due to the difficulty in controlling hypercortisolism by adrenal blocking drugs. Recent reports (including the initial follow-up of this patient) have suggested that tyrosine kinase inhibitors (TKIs) may be a therapeutic option due to an anti-secretory action on ACTH. However, there is a lack of long-term follow-up studies.

Dual Specificity Phosphatase 5, a Specific Negative Regulator of ERK Signaling, Is Induced by Serum Response Factor and Elk-1 Transcription Factor.

Serum stimulation of mammalian cells induces, via the MAPK pathway, the nuclear protein DUSP5 (dual-specificity phosphatase 5), which specifically interacts with and inactivates the ERK1/2 MAP kinases. However, molecular mechanisms underlying DUSP5 induction are not well known. Here, we found that the DUSP5 mRNA induction depends on a transcriptional regulation by the MAPK pathway, without any modification of the mRNA stability.

The Great Imitator in Endocrinology: A Painful Hypophysitis Mimicking a Pituitary Tumor.

Context: The incidence of syphilis has been increasing in recent decades in Western countries. Pituitary involvement is very unusual in syphilis. This infectious disease is not often considered in the workup of a patient with hypophysitis.

[Pregnancy and thyroid disorders].

Thyroid disorders are frequent among women, with a few specificities during pregnancy. Recent guidelines from the Endocrine Society concerning the management of thyroid disorders during pregnancy have been published, one year after the guidelines published by the American Thyroid Association. Iodine deficiency in France can increase the development of thyroid disorders during pregnancy.

[Premature ovarian failures].

Premature ovarian failure (POF) is clinically suspected by amenorrhea and confirmed by an elevated FSH serum level above 40 mUI/L (even 20 mUI/L) twice, in a woman before the age of 40. Prevalence of POF is between 1 to 2% in women. In 90% of cases, no aetiology is identified.

[Diagnosis and management of amenorrhea in adolescent girls].

Amenorrhea in adolescents can be primary, with or without breast development, or secondary. Whether amenorrhea is primary or secondary, height, body mass index, food intake, the level of physical activity per week, the presence of hirsutism or galactorrhea, pelvic pain and past history of intercourse need to be investigated. Initially, blood tests should include hCG, FSH, estradiol, testosterone and prolactin serum levels.

[Meno-metrorrhagia, dysmenorrhea in adolescents].

Menometrorrhagia is a common symptom in adolescents. It is idiopathic in most cases. In case of menometrorrhagia, it is necessary to exclude a pregnancy, a disorder of hemostasis, particularly the von Willebrand disease, as it represents the most common inherited disorder, and more rarely a chronic disease or an endocrinopathy.

Frequent large germline HRPT2 deletions in a French National cohort of patients with primary hyperparathyroidism.

Context: Hyperparathyroidism-jaw tumor syndrome (HPT-JT) is an autosomal dominant syndrome with incomplete penetrance that can associate in a single patient parathyroid adenoma or carcinoma, fibro-osseous jaw tumor, cystic kidney lesion, and uterine tumor. Germline mutations of the HRPT2 gene (CDC73) coding for parafibromin are identified in approximately 50%-75% of HPT-JT cases and in approximately 14% of familial isolated hyperparathyroidism. A whole deletion of this gene has recently been reported in 1 sporadic case and in a family presenting with HPT-JT.

A new contraceptive pill containing 17β-estradiol and nomegestrol acetate.

Most combined oral contraceptive pills contain ethinyl estradiol (EE) with progestins. In order to minimize the pill's cardiovascular risks, the concept of using 17β-estradiol (E2), the endogenous estradiol, arose in the 1970s. Many attempts to develop a pill containing 17β-E2 have failed as cycle control was low.

[Nonbacterial thrombotic endocarditis and gastric carcinoma].

We report a 74-year-old woman with acute heart failure and recurrent ischemic strokes as the presenting features of a nonbacterial thrombotic endocarditis complicating a gastric adenocarcinoma. The treatment only allowed a few months remission. Diagnosis of nonbacterial thrombotic endocarditis is rarely obtained while the patient is alive.

[New medical treatments in Cushing disease].

The standard treatment of Cushing disease is surgery. Radiotherapy and steroïdogenesis inhibitors are second-line treatments. The new multi-ligand somatostatin analogs, such as SOM230, having a much higher affinity with several receptors of somatostatin (SSTR1,3, and 5) than octreotid (mostly a SSTR2 receptor) have been synthetized.

[Endocannabinoid system: from metabolic to neuroendocrine effects].

The endocannabinoid system associates the cannabinoid receptors (CB1 and CB2), largely expressed in the central nervous system as well as in the peripheric one, the endocannabinoids (the two main ones being anandamine and the 2 arachidonoyl glycerol), the endocannabinoid-degrading enzymes, and the synthetic antagonists of the CB1 receptor, such as SR141716 (rimonabant). Mechoulam and his team have evidenced the neuroprotector role of endocannabinoids in case of cranial traumatism. As regards the hypothalamo-pituitary-gonadal axis, the administration of endocannabinoids in women would induce a lowering of the LH levels correlating with a loss of its pulsativity.