Hereditary medullary thyroid carcinoma (MTC) presents within the context of 3 genetic syndromes. Multiple endocrine neoplasia type 2A (MEN2A, Sipple’s syndrome) is an autosomal-dominant genetic syndrome that includes MTC, pheochromocytoma, and primary hyperparathyroidism (PHPT). MEN2B is an autosomal-dominant genetic syndrome that includes MTC, pheochromocytoma, multiple mucosal neuromas, and a marfanoid habitus. Familial medullary thyroid carcinoma (FMTC) refers to a variant of MEN2A in which MTC is the only clinical manifestation. These hereditary syndromes are uncommon; probably fewer than 1500 kindreds are recognized worldwide. MEN2 is estimated to affect 1 out of every 30,000-50,000 people. MEN2A is more common than FMTC, while MEN2B is the least common hereditary syndrome of the three.

Le pronostic de la NEM2 est théoriquement lié à l’âge de diagnostic du cancer médullaire thyroïdien. Des recommandations ont été faites pour uniformiser l’âge de la thyroïdectomie prophylactique. Notre objectif était d’analyser le devenir des patients NEM2 suivis dans notre centre et opérés après l’âge recommandé de thyroïdectomie (recommandations de l’American Thyroid Association : Wells et al., Thyroid, 2015). Étude monocentrique, rétrospective. Parmi 51 patients (RET Codon 533, n = 2 ; 618, n = 2 ; 634, n = 43 ; 883, n = 2 ; 918, n = 2) analysés, 6 (918, n = 1 ; 634, n = 5) opérés selon les recommandations sont en rémission. Sur les 45 patients opérés hors recommandations, 23 sont en rémission, dont 20 porteurs d’une mutation 634 et opérés en moyenne 12,7 ans après l’âge recommandé (min, 1 ; max, 43 ans après ; dont 10 opérés au moins 10 ans après). À l’inverse, 22 patients ne sont pas guéris dont 18 porteurs d’une mutation 634 et opérés en moyenne 17 ans après l’âge recommandé (min 1 ; max, 30 ans après ; âge moyen, p = 0,19 vs patients 634 en rémission ; calcitonine moyenne au dernier bilan à 900 pg/mL) : 4 patients porteurs d’une mutation 634, opérés 6, 8, 10 et 13 ans après l’âge recommandé sont métastatiques. Une fois passé l’âge recommandé pour la thyroïdectomie prophylactique, l’âge à la chirurgie ne préjuge pas du devenir du patient en termes de rémission ou agressivité de la pathologie résiduelle : chez ces patients, les chirurgies les plus tardives n’ont pas toujours le plus mauvais pronostic.

The management of hereditary pheochromocytoma has drastically evolved in the last 20 years. Bilateral pheochromocytoma does not increase mortality in MEN2 or von Hippel-Lindau (VHL) mutation carriers who are followed regularly, but these mutations induce major morbidities if total bilateral adrenalectomy is performed. Cortical sparing adrenal surgery may be proposed to avoid definitive adrenal insufficiency. The surgical goal is to leave sufficient cortical tissue to avoid glucocorticoid replacement therapy. This approach was achieved by the progressive experience of minimally invasive surgery via the transperitoneal or retroperitoneal route. Cortical sparing adrenal surgery exhibits <5% significant recurrence after 10 years of follow-up and normal glucocorticoid function in more than 50% of the cases. Therefore, cortical sparing adrenal surgery should be systematically considered in the management of all patients with MEN2 or VHL hereditary pheochromocytoma. Hereditary pheochromocytoma is a rare disease, and a randomized trial comparing cortical sparing vs classical adrenalectomy is probably not possible. This lack of data most likely explains why cortical sparing surgery has not been adopted in most expert centers that perform at least 20 procedures per year for the treatment of this disease. This review examined recent data to provide insight into the technique, its indications, and the results and subsequent follow-up in the management of patients with hereditary pheochromocytoma with a special emphasis on MEN2.

Post-traumatic diabetes and Graves-Basedow disease are entities, which are traditionally recognised by physician specialized in the compensation of personal injury. However, according to the scientific literature, the role played by stressful events as either triggering or precipitating of both autoimmune diseases remains hypothetic. Besides, if involved, the participation of stress could be only partial and indirect. Indeed, there is some disagreement between definite judicial precedents and scientific literature, which introduces some doubt. This review recalls that expert should take in account the imprecision of conclusions issued from scientific data to draw up its preconisation. He could only put forward a panel of presumptions in place of definite conclusion.

L’objectif de cette étude était d’évaluer la pertinence du test couplé déxaméthasone desmopressine (TCDD) comme facteur prédictif de récidive de maladie de Cushing après chirurgie trans-sphénoïdale. Nous avons rétrospectivement étudié 67 patients dans deux centres tertiaires (Marseille et Grenoble), en rémission initiale après chirurgie transsphénoïdale et suivis au moins 18 mois. Nous avons évalué à 3 mois puis annuellement le cortisol libre urinaire sur 24 h, les taux d’ACTH et de cortisol plasmatiques, la réponse aux tests de freinage minute et à la desmopressine et le TCDD. Après TCDD, un ratio d’ACTH (ACTHr) et de cortisol (cortisolr) étaient déterminés par la formule (pic – valeur basale)/valeur basale. Onze patients avaient récidivé après un suivi médian de 52 mois (18–180). L’association ACTHr et cortisole supérieur ou égal à 0,5 était présente chez tous les patients ayant récidivé et chez 14/56 patients en rémission (sensibilité = 100 %, spécificité = 75 %). La positivité du TCDD précédait de 6-76 mois les marqueurs classiques d’hypercorticisme chez 10/11 patients ayant récidivé. Six patients avaient récidivé malgré une cortisolémie à 8 h en postopératoire immédiat inférieur à 50 nmol/L : tous avaient un TCDD positif dans les 3 ans suivant la chirurgie. La réponse au TCDD était reproductible au cours des évaluations chez 91 % des patients. Le TCDD est un facteur prédictif précoce et fiable de récidive de maladie de Cushing et a un intérêt majeur dans les 3 ans postopératoire pour optimiser la surveillance à long terme.

Le capital folliculaire est constitue d’un stock definitif et genetiquement determine de cellules germinales formees au cours de la vie embryonnaire et fœtale pour la totalite de la vie reproductive. Ce capital folliculaire diminue progressivement des le cinquieme mois de developpement jusqu’a la menopause, par des phenomenes d’atresie et d’ovulation. Ainsi, la fonction ovarienne d’une femme depend a la fois du capital folliculaire initialement forme, mais aussi de la cinetique de l’atresie folliculaire. L’atresie folliculaire est la consequence d’actions entre des genes proapoptotiques et antiapoptotiques. Grâce aux etudes d’invalidation genetique sur modeles animaux et a celles menees chez des patientes atteintes de dysgenesie gonadique et d’insuffisance ovarienne prematuree, il a ete montre que la constitution de la reserve ovarienne folliculaire etait genetiquement determinee, et ce des la formation de la gonade. Bien qu’aucun gene-cle de la differenciation ovarienne, homologue a sex determining region Y (SRY) pour le testicule, n’ait ete decrit chez la femme, il existe cependant une cascade genetique comportant des genes antitesticulaires, ainsi que des genes pro-ovariens conduisant a la differenciation de la gonade bipotentielle en ovaire comme les genes WNT4, R spondine1 (RSPO) et DAX1. L’insuffisance ovarienne prematuree peut etre la consequence : soit d’un defaut initial de formation des follicules primordiaux par anomalie d’un des genes de la cascade genetique du determinisme gonadique, soit d’une atresie folliculaire acceleree, comme par exemple dans les anomalies de nombre ou de structure du chromosome X, soit d’un blocage de la maturation folliculaire, comme par exemple en cas de mutation du gene de recepteur a la follicle stimulating hormone (FSH). L’amelioration des connaissances concernant le controle genetique du capital folliculaire offre des pistes de recherche pour traiter les patientes atteintes ou a risque d’insuffisance ovarienne precoce du fait de traitements gonadotoxiques : utilisation de facteurs antiapoptotiques, therapie genique.

Context: Fluorine-18-L-dihydroxyphenylalanine positron emission tomography (18F-FDOPA PET) imaging is increasingly used in the workup of neuroendocrine tumors. It has been shown to be an accurate tool in the diagnosis of congenital hyperinsulinism, but limited information is available on its value in adult disease. Objective, Patients, and Design: The objective of this study was to review our experience with 18F-FDOPA PET imaging in six consecutive patients with hyperinsulinemic hypoglycemia (HH) (four solitary insulinomas, one diffuse beta-cell hyperplasia, one malignant insulinoma). 18F-FDOPA uptake was also evaluated in 37 patients (43 procedures) without HH or other pancreatic neuroendocrine tumors, which acted as a control group. Results: Using visual analysis, 18F-FDOPA-PET proved positive in only one case (a multiple endocrine neoplasia type 1 related insulinoma). In diffuse beta-cell hyperplasia, the pancreatic uptake was similar to controls. In the patient with liver metastases, the extent of disease was underestimated. The pancreatic uptake was not statistically different between controls and hyperinsulinemic patients. The main limitation for identifying insulinomas or beta-cell hyperplasia in adults appears to be to the 18F-FDOPA uptake and retention in the whole pancreas. This drawback is potentially circumvented in focal hyperplasia in newborns due to a lower aromatic amino acid decarboxylase expression in the extralesional pancreatic parenchyma. Conclusions: 18F-FDOPA PET is of limited value in localizing pancreatic insulin secreting tumors in adult HH. Our results contrast with the referential study and require further analysis. (J Clin Endocrinol Metab 95: 303-307, 2010)

But de l’étude : Le but de cette étude prospective était d’évaluer les bénéfices et les contraintes de la cervicotomie, de l’abord électif et de la vidéochirurgie dans le traitement de l’hyperparathyroïdie primaire (HPT 1).Patients et méthodes : Au cours de l’année 1998, 66 patients ont été opérés pour HPT 1 dans le même centre. Il s’agissait de 48 femmes et 18 hommes (âge moyen : 58 ans, extrêmes : 21–84). Aucun n’avait de forme familiale ou de néoplasie endocrinienne multiple. L’intervention a été réalisée par cervicotomie (n : 32), par abord électif latéralisé (n = 8), par vidéochirurgie (n = 25). Un adénome médiastinal a été enlevé par cervicotomie et un autre par thoracoscopie gauche. Le dosage rapide de la parathormone (PTH) était effectué 20 minutes après exérèse de l’adénome. La calcémie était dosée à la 24e et 48e heure et deux mois après l’intervention.Résultats : L’examen anatomopathologique a trouvé un adénome double et 65 adénomes uniques. L’exérèse a été suivie dans tous les cas d’une chute significative de la PTH. Dans le groupe traité par chirurgie vidéo-assistée, il y a eu 11 conversions en cervicotomie (44 %) et une paralysie récurrentielle. À la fin de l’étude, tous les patients sauf un étaient normocalcémiques.Conclusion : Ce travail confirme la faisabilité de la chirurgie vidéo-assistée dans l’hyperparathyroïdie. Elle nécessite un repérage préopératoire de l’adénome et un dosage rapide peropératoire de la PTH. Elle permet de réduire la taille de la cicatrice et évite la sternotomie pour l’exérèse des adénomes médiastinaux. L’abord électif a un intérêt comparable à celui de la vidéochirurgie et une plus grande simplicité. La cervicotomie conserve une place de choix en cas de cervicotomie antérieurement, de thyroïdectomie associée, de non repérage de l’adénome par l’imagerie et chez les sujets âgés moins concernés par des préoccupations esthétiques.Study aim: The aim of this prospective study was to assess the advantages and disadvantages of cervicotomy, selective lateral approach and video-assisted surgery in the treatment of primary hyperparathyroidism (HPT 1).Patients and methods: During 1998, 66 patients were operated on for HPT 1 in the same center. There were 48 women and 18 men (mean age : 58 years, range : 21–84), familial HPT 1 or MEN1 excluded. The interventions were performed via classical cervicotomy (n = 32), via selective lateral approach (n = 8) and were video-assisted (n = 25). A mediastinal adenoma was removed via cervicotomy and another one via left thoracoscopy. The procedure was associated with intraoperative parathormone (PTH) quick-assay. Calcium testing was controlled before leaving the hospital and 2 months later.Results: A double adenoma and 65 single adenomas were confirmed by pathological report. Circulating PTH levels, 20 minutes after removal of the adenoma, always decreased significantly. In video-assisted procedures, there were 11 conversions to open cervicotomy (44 %) and morbidity consisted of one case of laryngeal nerve paralysis. At the end of the study, all patients except one had a normal calcium level.Conclusion: Video-assisted parathyroidectomy is feasible but requires a preoperative localisation of the adenoma and intraoperative PTH quick-assay. Its main benefit in cervical adenoma is to reduce the scar and in mediastinal adenoma to avoid sternotomy. With the elective approach, results are similar to those of video-assisted surgery and the procedure is much easier to perform. Classical cervicotomy is still the best option in case of previous cervicotomy, of simultaneous thyroidectomy, of negative preoperative imaging and in elderly patients less concerned about cosmetic benefit.

Twenty-five percent of medullary thyroid cancers (MTC) are familial and inherited as an autosomal dominant trait. Three different phenotypes can be distinguished: multiple endocrine neoplasia (MEN) types 2A and 2B, in which the MTC is associated with other endocrine neoplasias, and familial MTC (FMTC), which occurs in isolation. The discovery that germline RET oncogene activating mutations are associated with 95-98% of MEN 2/FMTC syndromes and the availability of genotyping to identify mutations in affected patients and their relatives has revolutionized the diagnostic and therapeutic strategies available for the management of these patients. All patients with MTC, both those with a positive familial history and those apparently sporadic, should be submitted to RET genetic screening. Once an RET mutation has been confirmed in an index patient, first-degree relatives should be screened rapidly to identify the 50% who inherited the mutation and are therefore at risk for development of MTC. Relatives in whom no RET mutation is identified can be reassured and discharged from further follow-up, whereas RET-positive subjects (i.e. gene carriers) must be investigated and a therapeutic strategy initiated. These guideline recommendations are derived from the most recent studies identifying phenotype-genotype correlations following the discovery of causative RET gene mutations in MEN 2 eighteen years ago. Three major points will be discussed: (a) identification of patients and relatives who should have genetic screening for RET mutations, (b) management of asymptomatic gene carriers, and (c) ethics.

Carcinome hypophysaire et néoplasie endocrinienne multiple de type 1 : efficacité à long-terme du temozolomide Melanie Philippon a, Isabelle Morange a, Marilyne Barrie b, Anne Barlier a,c, David Taieb d, Henry Dufour e, Bernard Conte-Devolx a, Thierry Brue a, Frédéric Castinetti a,∗ a Department of endocrinology and reference center for rare pituitary diseases, hôpital Timone, assistance publique hôpitaux de Marseille, Aix-Marseille université, 13284 Marseille, France b Department of neurology and oncology, hôpital Timone, assistance publique hôpitaux de Marseille, Aix-Marseille université, 13284 Marseille, France c Department of molecular biology, hôpital La Conception, assistance publique hôpitaux de Marseille, Aix-Marseille université, 13284 Marseille, France d Department of nuclear medicine, hôpital Timone, assistance publique hôpitaux de Marseille, Aix-Marseille université, 13284 Marseille, France e Department of neurosurgery, hôpital Timone, Aix-Marseille université, 13284 Marseille, France

Purpose of review Mifepristone is the first and only available glucocorticoid receptor antagonist. Cushing's syndrome is a rare disease, responsible for increased morbidity and mortality. The treatment of Cushing's syndrome is far from perfect. The aim of this review is to better define the merits and pitfalls of mifepristone in treating Cushing's syndrome, and try to determine its potential roles in the treatment of hypercortisolism. Recent findings Only case reports or series based on a low number of patients had been reported in the literature. A recent retrospective European Study based on about 20 patients with Cushing's syndrome gave more precise data about mifepristone. Coupled with the 20 previously reported patients in various studies, these results determine the profile of patients who could benefit from mifepristone. Summary High clinical efficacy of mifepristone is counterbalanced by the lack of available biological monitoring data during treatment, and the risk of worsening of hypokalemia and blood pressure levels. However, its rapid efficacy and the fact that the drug uses a mechanism that is different from all currently available treatment should make mifepristone a valuable treatment in particular cases of uncontrolled hypercortisolism despite classical methods.

Une patiente de 52 ans présentant une aménorrhée secondaire a été hospitalisée en raison de la survenue d'une ophtalmoplégie et d'une hémorragie sousarachnoïdienne. L'exploration endocrinienne des différents axes anté-hypophysaires montrait une hyperprolactinémie modérée et un déficit des autres fonctions thyréotrope, gonadotrope, corticotrope et somatotrope. La radiographie du crâne révélait un agrandissement de la selle turcique; l'examen tomodensitométrique était en faveur d'une tumeur hypophysaire. L'artériographie carotidienne confirmait l'existence d'un anévrysme de la carotide interne droite, en position intrasellaire, sans formation tumorale associée. Après embolisation de l'anévrysme, suivie d'une anastomose temporo-sylvienne droite, le bilan endocrinien restait inchangé. Le mécanisme de l'hyperprolactinémie, probablement due à une ischémie hypophysaire, est discuté. Cette observation permet d'insister sur la nécessité d'une exploration neuroradiologique précise avant l'exérèse d'un adénome hypophysaire, d'autant qu'il s'y associe des signes d'hémorragie méningée.A 52-year-old woman with secondary amenorrhea presented with ophtalmoplegia, subarachnoidal bleeding. Pituitary function tests showed mild hyperprolactinemia and deficiencies of other functions of adenohypophysis. X-ray films of the skull showed enlarged sella turcica, and CT scan was interpreted as demonstrating pituitary tumour. Carotid arteriography led to diagnosis of intrasellar aneurysm of the right internal carotid, without any pituitary tumour. After embolisation of the aneurysm, followed, by a temporo-sylvian anastomosis, endocrine functions did not improve. The mechanism of hyperprolactinemia is discussed, probably due to pituitary ischemia. This case provides evidence of interest of further investigations before a transsphenoidal surgery in pituitary tumours, in particular if subarachnoidal bleeding occurs.

Le traitement du diabète de type 2 (DT2) doit être précoce, comportant des mesures hygiénodiététiques indispensables mais insuffisantes pour contrôler seules la dérive glycémique. Des antidiabétiques oraux (ADO) et l’insuline ont fait la preuve d’une efficacité métabolique et de prévention des complications. Toutes les recommandations suggèrent la mise initiale sous metformine, puis le passage sans retard en bithérapie, voire trithérapie, avec les molécules existantes (sulfamides hypoglycémiants d’abord, thiazolidinediones [TZD] ensuite), puis un passage à l’insuline. Les nouveaux agents antidiabétiques, maintenant disponibles, agissent en amplifiant la réponse insulinique, phénomène dit « incrétine », dû à l’action d’un peptide digestif, le GLP1, qui stimule de façon glucodépendante les cellules β du pancréas endocrine. Le GLP1 est diminué dans le DT2, expliquant pour partie les troubles de l’insulinosécrétion, l’élévation basale et le non-freinage postprandial du glucagon, l’excès de production hépatique de glucose et ainsi l’élévation des glycémies à jeun et postprandiales. Le GLP1 a une demi-vie très courte, car rapidement dégradé par des enzymes ubiquitaires, les DPP4. GLP1 est trophique pour la cellule β, agit sur la satiété et la vidange gastrique. Sa demi-vie est accrue par l’administration orale d’inhibiteurs des DPP4 ou gliptines (sitagliptine et vildaglpitine). Ces agents abaissent la glycémie (diminution de 0,5 à 1,1 % de l’HbA1c), l’effet portant en premier lieu sur les glycémies postprandiales mais également présent sur les glycémies nocturnes, à jeun et interprandiales. Ils ont peu d’effets indésirables et ne s’accompagnent d’aucune prise de poids ni d’hypoglycémie. L’autre approche est représentée par l’injection sous-cutanée d’analogues du GLP1 résistants aux DPP4 : l’exenatide et le liraglutide. L’effet hypoglycémiant est plus marqué (diminution de 0,5 à 1,7 % de l’HbA1c), le poids diminue de quelques kilos, mais les taux de GLP1 pharmacologiques induits s’accompagnent de plus d’effets indésirables, sans risque d’hypoglycémie toutefois. Les deux types d’agents (gliptines) sont en particulier indiqués en bithérapie avec la metformine, voire les TZD. Ces nouvelles molécules ouvrent de nouvelles perspectives dans le traitement médicamenteux du DT2 qui sont ici discutées.Treatment of type 2 diabetes (T2DM) is based on lifestyle changes and oral antidiabetic agents or insulin. The UKPDS study has confirmed metformin (Met) as the initial monotherapy. Accordingly, Met is widely regarded as the first drug of choice for most patients with T2DM. Safety and efficacy of sulphonylureas (SU) have been confirmed by several clinical trials. Recently, thiazolidinediones (TZD) have addressed some aspects of insulin-resistance that characterized several T2DM patients. However, SU and TZD are associated with various side effects that limit their use in many patients. New agents have been recently developed which potentiate the activity of the incretin (GLP1). GLP1, a gut hormone secreted in response to meal ingestion, is rapidly degraded by dipeptidylpeptidase-4 (DPP-4). GLP1 enhances insulin secretion and inhibits glucagon secretion in a glucose-dependent manner, delays gastric emptying and, in animal studies, preserves β-cell mass by reducing apoptosis and stimulates of β-cell proliferation. GLP1 levels are abnormally low in T2DM patients. Two classes of agents based on GLP1 have been launched: DPP-4 inhibitors and DPP-4 resistant GLP1 analogues. Randomized studies confirmed their efficacy to improve glycemic control in T2DM patients. Orally administered DPP-4 inhibitors reduce HbA1c by 0.5–1.1%, without hypoglycaemic events and no weight gain. The sub-cutaneous injected GLP1 analogues (exenatide and liraglutide) show larger reductions in HbA1c by 0.8–1.7% and weight loss but are associated with gastrointestinal side effects contributing to a significant treatment interruption. Several studies support the use of DPP-4 inhibitors in combination with Met as a promising second line treatment.

CONTEXT:A few prospective studies have evaluated the use of recombinant human TSH (rhTSH) for radioiodine remnant ablation.OBJECTIVE:Our objective was to compare the effects of the both TSH regimens on iodine biokinetics in the thyroid remnant, dosimetry, and radiation protection.DESIGN:We conducted a prospective randomized study.MATERIALS AND METHODS:Eighty-eight patients were enrolled for radioiodine ablation to either the hypothyroid or rhTSH arms. A whole-body scan was performed at 48 and 144 h after therapy. Dose rates were assessed at 24, 48, and 144 h. Urinary samples were obtained during the first 48 h. Thyroglobulin was assessed before and after therapy. Iodine biokinetics in the remnants were calculated from gamma-count rates. Radiation-absorbed dose was calculated using OLINDA software. Exposure estimation was based on a validated model.RESULTS:The effective half-life in the remnant thyroid tissue was significantly longer after rhTSH than during hypothyroidism (P = 0.01), whereas 48-h (131)I uptakes and residence times were similar. After therapy, thyroglobulin release (a marker of cell damage) was lower in the rhTSH arm. The mean total-body effective half-life and residence time were shorter in patients treated after rhTSH. Residence time was also lower for the colon and stomach. Absorbed dose estimates were lower in the rhTSH arm for the lower large intestine, breasts, ovaries, and the bone marrow. Dose rates at the time of discharge were lower in the rhTSH group with a reduction in cumulative radiation exposure to contact persons.CONCLUSIONS:In comparison with thyroid hormone withdrawal, rhTSH is associated with longer remnant half-life of radioactive iodine while also reducing radiation exposure to the rest of the body and also to the general public who come in contact with such patients.

Multiple endocrine neoplasia type 1 (MEN1) and type 2 (MEN2) are autosomal dominant inherited multiglandular diseases with familial and individual age-related penetrance and variable expression. The most frequent endocrine features of MEN1 are parathyroid involvement (> 95%), duodeno-pancreatic endocrine tissue involvement (80%), pituitary adenoma (30%), and adrenal cortex tumors (25%), with no clear syndromic variants. Identification of the germline MEN1 mutation confirms the diagnosis, but there is no phenotype-genotype correlation. All patients with MEN2 have medullary thyroid carcinoma (MTC). The most distinctive MEN2 variants are MEN2A (MTC+pheochromocytoma+hyperparathyroidism), MEN2B (MTC+pheo), and isolated familial MTC (FMTC). The prognosis of MEN2 is linked to the progression of MTC, which depends mainly on the stage at diagnosis and the quality of initial surgical treatment. This emphasizes the need for early diagnosis and management. The specific RET codon mutation correlates with the MEN2 syndromic variant and with the age of onset and aggressiveness of MTC. Consequently, RET mutational status should guide major management decisions, such as whether and when to perform thyroidectomy.

Aim: Our objective was to report a single-center experience of the management of pituitary tumor apoplexy. Patients and methods: We retrospectively analyzed a series of 44 patients hospitalized for pituitary apoplexy between January 1996 and March 2008 at the Timone Hospital, Marseille, France. Results: Most frequent presenting symptoms were headaches (93%), visual impairment (85%) and vomiting (59%). Hypopituitarism was present at diagnosis in 88% of patients, with a high incidence of corticotroph deficiency (70%). A risk factor was found in 52% of patients, mostly hypertension. Apoplexy occurred in a previously undiagnosed pituitary adenoma in 32/44 cases (73%). The apoplectic event concerned 12 secreting, 27 non-functioning, 4 uncharacterized adenomas and one Rathke’s pouch cyst. Nineteen patients underwent surgery within the first month, and one patient had conventional radiotherapy. Twenty-four patients, who had no ophthalmic or neurological signs, were conservatively treated in first intention; among them, 6 received high dose corticosteroids. After a median follow-up of 21 months, there was no significant difference in terms of endocrine or visual recovery between the operated and the conservatively treated groups, nor between patients treated with corticosteroids or not. Panhypopituitarism was observed in 52% of patients, but partial or complete visual recovery was present in the majority of patients (91%), whatever the therapeutic approach. Conclusion: The outcome of patients treated with or without surgery for pituitary apoplexy without severe neuro-ophthalmic deficits seems to be identical, pleading for a conservative management of pituitary apoplexy in the absence of visual emergency.

The widespread use of high-resolution cross-sectional imaging such as computed tomography (CT) and magnetic resonance imaging (MRI) for the investigation of the abdomen is associated with an increasing detection of incidental adrenal masses. We evaluated the ability of 18F-fluorodeoxyglucose positron emission tomography to distinguish benign from malignant adrenal masses when CT or MRI results had been inconclusive.

IgE(ND) was labelled with 125I to various levels of radioactivities. Changes in the amount fo immunoreactive material as well as the immunoreactivity of the different 125I-IgE(ND) preparations were studied during 1 year period of storage. The labelled product can be used for at least 5·5 months for the radioimmunoassay of IgE and up to 1 year if prolonged counting times, up to 1 hr per test tube, are used.

OBJECTIVE:Mifepristone is the only available glucocorticoid receptor antagonist. Only few adult patients with hypercortisolism were treated to date by this drug. Our objective was to determine effectiveness and tolerability of mifepristone in Cushing's syndrome (CS).DESIGN:Retrospective study of patients treated in seven European centers.METHODS:Twenty patients with malignant (n=15, 12 with adrenocortical carcinoma, three with ectopic ACTH secretion) or benign (n=5, four with Cushing's disease, one with bilateral adrenal hyperplasia) CS were treated with mifepristone. Mifepristone was initiated with a median starting dose of 400 mg/day (200-1000). Median treatment duration was 2 months (0.25-21) for malignant CS, and 6 months (0.5-24) for benign CS. Clinical (signs of hypercortisolism, blood pressure, signs of adrenal insufficiency), and biochemical parameters (serum potassium and glucose) were evaluated.RESULTS:Treatment was stopped in one patient after 1 week due to severe uncontrolled hypokalemia. Improvement of clinical signs was observed in 11/15 patients with malignant CS (73%), and 4/5 patients with benign CS (80%). Psychiatric symptoms improved in 4/5 patients within the first week. Blood glucose levels improved in 4/7 patients. Signs of adrenal insufficiency were observed in 3/20 patients. Moderate to severe hypokalemia was observed in 11/20 patients and increased blood pressure levels in 3/20 patients.CONCLUSION:Mifepristone is a rapidly effective treatment of hypercortisolism, but requires close monitoring of potentially severe hypokalemia, hypertension, and clinical signs of adrenal insufficiency. Mifepristone provides a valuable treatment option in patients with severe CS when surgery is unsuccessful or impossible.