Phéochromocytome (une nouvelle complication du syndrome d'Eisenmenger?)

Les phéochromocytomes sont des tumeurs neuroendocrines rares sécrétant des catécholamines. Le syndrome d'Eisenmenger est une pathologie également rare, qui représente la forme la plus évoluée d'hypertension artérielle pulmonaire associée à une cardiopathie congénitale cyanogène. Les patients atteints du syndrome d'Eisenmenger sont à haut risque de développer des complications médicales, liées à l'hypoxémie chronique notamment. De récents progrès médicaux et chirurgicaux ont amélioré significativement leur espérance de vie. Puisque leur survie jusqu'à l'âge adulte est désormais plus fréquente, la découverte de nouvelles complications est attendue. La survenue d un phéochromocytome serait une de ces nouvelles complications. Nous rapportons ici un cas de phéochromocytome associé à un syndrome d'Eisenmenger. A notre connaissance, 25 cas de phéochromocytomes associés à une cardiopathie congénitale ont déjà été décrits. La découverte de la coexistence de ces deux maladies rares chez plusieurs individus suggère une association non fortuite. Parmi les hypothèses évoquées, l'hypoxémie chronique ou des anomalies génétiques pourraient favoriser la survenue d un phéochromocytome dans ce contexte. Ce cas instructif nous incite à considérer la possibilité d un phéochromocytome chez les sujets porteurs d un syndrome d'Eisenmenger. C'est pourquoi, outre la classique triade de Ménard, l'aggravation de l'insuffisance cardiaque ou la survenue de symptômes inattendus doit faire suspecter le diagnostic de phéochromocytome chez tout patient atteint de cardiopathie congénitale cyanogène.NANTES-BU Médecine pharmacie (441092101) / SudocSudocFranceF

Testosterone-induced acne fulminans in twins with Kallmann's syndrome

International audienceno abstrac

Механизм туристской ренты как инструмент стимулирования развития туристского продукта малых городских поселений Беларуси

В сборнике материалов конференции отражены научно-методические и прикладные результаты научных исследований, анализа, оценки, регулирования, картографического и ГИС-обеспечения современных структурных и региональных сдвигов в мировом хозяйстве, социально-экономической модернизации стран и регионов СНГ в условиях глобализации, инновационных факторов социально-экономического развития регионов Беларуси, демографического развития и социально-демографических рисков стран, современных региональных проблем развития туризма, природно-ресурсного потенциала стран и регионов, геоэкологических аспектов стратегии устойчивого развития. Адресуется преподавателям, научным работникам, студентам и аспирантам вузов, сотрудникам органов управления

The NFAT3/RERG Complex in Luminal Breast Cancers Is Required to Inhibit Cell Invasion and May Be Correlated With an Absence of Axillary Lymph Nodes Colonization

International audienceLuminal breast cancers represent 70% of newly diagnosed breast cancers per annum and have a relatively good prognosis compared with triple-negative breast cancers. Luminal tumors that are responsive to hormonal therapy are particularly associated with a favorable prognosis. Nonetheless, the absolute number of metastatic relapses in luminal cancers is larger than in triple-negative breast cancers. A better understanding of the biology of luminal cancers, control of metastases formation, and identification of predictive markers of their evolution are therefore still necessary. In this context, we previously disclosed the key role of NFAT3 in regulating luminal breast cancer invasion. We have now identified a specific inhibitory region, in the C-terminal part of NFAT3, required for the inhibition of invasion of the human luminal breast cancer cell line T-47D. Indeed, we showed that this 85 amino acid C-terminal region acts as a dominant negative form of NFAT3 and that its overexpression in the T-47D cell line led to increased cell invasion. Mechanistically, we have revealed that this region of NFAT3 interacts with the small Ras GTPase RERG (RAS like estrogen regulated growth inhibitor) and shown that RERG expression is required for NFAT3 to impede T-47D cell invasion. We have validated the association of NFAT3 with RERG in human luminal breast cancer tissues. We have shown an increase of the quantity of the NFAT3/RERG complexes in patients without axillary lymph node colonization and therefore proposed that the detection of this complex may be a non-invasive marker of axillary lymph node colonization

Head-to-Head Comparison of 18F-DOPA PET/CT and 68Ga-DOTANOC PET/CT in Patients With Midgut Neuroendocrine Tumors

International audiencePurpose: The aim of this study was to compare retrospectively F-DOPA PET/CT versus Ga-DOTANOC PET/CT in a group of patients affected by midgut NET.Patients and methods: Patients with histologically proven grade 1 or grade 2 midgut NET were explored after injection of 150 MBq of Ga-DOTANOC and 210 MBq of F-DOPA. The PET/CTs were analyzed visually and semiquantitatively at the patient level, regional level (7 defined regions), and lesion level (maximum of 5 lesions/organ). The criterion standard was determined on the basis of histology and imaging follow-up.Results: Thirty patients (17 males and 13 females; median age, 63.5 years [37-82 years]) were included. Both PET/CTs were negative in 3 patients and positive in 25 patients. PET/CTs were discordant in 2 patients, with F-DOPA positive and Ga-DOTANOC negative. F-DOPA PET/CT detected more involved regions and more metastatic lesions than Ga-DOTANOC PET/CT in 6 (20%) and 10 (33.3%) patients, respectively. Of the 81 confirmed affected regions, 77 (95%) were detected by F-DOPA PET/CT and 71 (87.7%) by Ga-DOTANOC PET/CT (P < 0.0001). F-DOPA PET/CT detected significantly more lesions (211/221) than Ga-DOTANOC PET/CT (195/221), corresponding to a sensitivity of 95.5% and 88.2%, respectively (P < 0.0001). Tumor-to-background ratios were more favorable in liver for F-DOPA than for Ga-DOTANOC. Interestingly, a correlation was found between F-DOPA SUVmax and tumor burden and especially with the number of regions involved by the disease (P = 0.019).Conclusions: F-DOPA PET/CT is superior to Ga-DOTANOC PET/CT for the detection of lesions, and when available, this tracer may be recommended as the first-line examination for an accurate staging of midgut NET

Prospective Multicentric Assessment of ⁶⁸Ga-DOTANOC PET/CT in Grade 1-2 GEP-NET

The aim of this multicentric study was to prospectively compare 68Ga-DOTANOC PET/CT versus somatostatin receptor scintigraphy (SRS) with SPECT/CT, combined with multiphasic CT scan and MRI in patients with grade 1 or 2 gastroenteropancreatic neuroendocrine tumors (GEP-NET). Patients with histologically proven grade 1 or 2 GEP-NET with suspicion of recurrence or progression, or with typical aspects of GEP-NET on morphological imaging, were explored with conventional imaging (CI): SRS with SPECT/CT, multiphasic CT scan and/or liver MRI followed by 68Ga-DOTANOC PET/CT. The gold standard was based on histology and imaging follow-up. The data of 105 patients (45 woman and 60 men; median age) were analyzed. 68Ga-DOTANOC PET/CT sensitivity was significantly higher than CI sensitivity in per-patient (98.9% vs. 88.6%, p = 0.016) and per-region (97.6% vs. 75.6%, p p = 0.016), peritoneal carcinomatosis (95% vs. 30%, p p = 0.041). 68Ga-DOTANOC PET/CT had an impact on the therapeutic management of 41.9% (44/105) patients compared to decisions based on CI explorations. Our data confirm the superiority of 68Ga-DOTANOC PET/CT over CI in the detection of peritoneal carcinomatosis and bone metastasis, as well as its strong therapeutic impact on the management of patients with grade 1-2 GEP-NETs

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

International audienc

Long-term Follow-up of MEN1 Patients Who Do Not Have Initial Surgery for Small ≤2 cm Nonfunctioning Pancreatic Neuroendocrine Tumors, an AFCE and GTE Study

IF 9.203International audienceObjective: To report long-term follow-up of patients with multiple endocrine neoplasia type 1 (MEN1) and nonfunctioning pancreatic neuroendocrine tumors (NF-PET).Background: Pancreaticoduodenal tumors occur in almost all patients with MEN1 and are a major cause of death. The natural history and clinical outcome are poorly defined, and management is still controversial for small NF-PET.Methods: Clinical outcome and tumor progression were analyzed in 46 patients with MEN1 with 2 cm or smaller NF-PET who did not have surgery at the time of initial diagnosis. Survival data were analyzed using the Kaplan-Meier method.Results: Forty-six patients with MEN1 were followed prospectively for 10.7 ± 4.2 (mean ± standard deviation) years. One patient was lost to follow-up and 1 died from a cause unrelated to MEN1. Twenty-eight patients had stable disease and 16 showed significant progression of pancreaticoduodenal involvement, indicated by increase in size or number of tumors, development of a hypersecretion syndrome, need for surgery (7 patients), and death from metastatic NF-PET (1 patient). The mean event-free survival was 13.9 ± 1.1 years after NF-PET diagnosis. At last follow-up, none of the living patients who had undergone surgery or follow-up had evidence of metastases on imaging studies.Conclusions: Our study shows that conservative management for patients with MEN1 with NF-PET of 2 cm or smaller is associated with a low risk of disease-specific mortality. The decision to recommend surgery to prevent tumor spread should be balanced with operative mortality and morbidity, and patients should be informed about the risk-benefit ratio of conservative versus aggressive management when the NF-PET represents an intermediate risk

Long-term Follow-up of MEN1 Patients Who Do Not Have Initial Surgery for Small ≤2 cm Nonfunctioning Pancreatic Neuroendocrine Tumors, an AFCE and GTE Study: Association Francophone de Chirurgie Endocrinienne &amp; Groupe d'Etude des Tumeurs Endocrines

To report long-term follow-up of patients with multiple endocrine neoplasia type 1 (MEN1) and nonfunctioning pancreatic neuroendocrine tumors (NF-PET)