Search our Database of Scientific Publications and Authors

I’m looking for a

    2275 results match your criteria Wermer Syndrome MEN Type 1

    1 OF 46

    MEN1 mutations and potentially MEN1-targeting miRNAs are responsible for menin deficiency in sporadic and MEN1 syndrome-associated primary hyperparathyroidism.
    Virchows Arch 2017 Jun 8. Epub 2017 Jun 8.
    "Lendület" Hereditary Endocrine Tumours Research Group, Hungarian Academy of Sciences, Semmelweis University, Szentkirályi utca 46, Budapest, H-1088, Hungary.
    Inherited, germline mutations of menin-coding MEN1 gene cause multiple endocrine neoplasia type 1 (MEN1), while somatic MEN1 mutations are the sole main driver mutations in sporadic primary hyperparathyroidism (PHPT), suggesting that menin deficiency has a central role in the pathogenesis of PHPT. MiRNAs are small, noncoding RNAs posttranscriptionally regulating gene expression. Our aim was to investigate both the role of MEN1 mutations and potentially MEN1-targeting miRNAs as the underlying cause of menin deficiency in MEN1-associated and sporadic PHPT tissues. Read More

    Laparoscopic total pancreatectomy for multiple endocrine neoplasia type 1 syndrome-associated multifocal, non-functioning pancreatic neuroendocrine tumor: A case report.
    Asian J Endosc Surg 2017 Jun 6. Epub 2017 Jun 6.
    Department of Hepatobiliary and Pancreatic Surgery, Gem Hospital and Research Centre, Coimbatore, Tamil nadu, India.
    Pancreatic neuroendocrine tumors are rare, accounting for less than 3% of all pancreatic tumors. Although laparoscopic pancreas-preserving surgery for managing sporadic pancreatic neuroendocrine tumors has been described in the literature, laparoscopic total pancreatectomy has rarely been reported. We present a 30-year-old man who was incidentally diagnosed with multiple endocrine neoplasia type 1 syndrome with parathyroid hyperplasia and a non-functioning pancreatic neuroendocrine tumor. Read More

    Pancreatic neuroendocrine tumors in MEN1 disease: a mono-centric longitudinal and prognostic study.
    Endocrine 2017 May 31. Epub 2017 May 31.
    Department of Endocrinology, Catholic University of the Sacred Heart, Rome, Italy.
    Purpose: Multiple endocrine neoplasia type 1 (MEN1) is an inherited endocrine neoplastic syndrome associated with a greater risk of endocrine tumor development like pancreatic neuroendocrine tumors (p-NET), with different clinical characteristics from sporadic ones. This paper aims to compare clinical, hystological and morphological aspects of p-NET in patients affected from MEN1 (MEN1+) and not-affected ones (MEN1-).

    Methods: We performed a retrospective observational study. Read More

    Challenging Differential Diagnosis of Hypergastremia and Hyperglucagonemia with Chronic Renal Failure: Report of a Case with Multiple Endocrine Neoplasia Type 1.
    Intern Med 2017 1;56(11):1375-1381. Epub 2017 Jun 1.
    Department of Diabetes and Endocrinology, Osaka Red Cross Hospital, Japan.
    A 53-year-old woman developed end-stage renal failure during a 15-year clinical course of primary hyperparathyroidism and was referred to our hospital for evaluation of suspected multiple endocrine neoplasia type 1 (MEN1). Genetic testing revealed a novel deletion mutation at codon 467 in exon 10 of the MEN1 gene. Systemic and selective arterial calcium injection (SACI) testing revealed hyperglucagonemia and hypergastrinemia with positive gastrin responses. Read More

    Sporadic Insulinoma Presenting as Early Morning Night Terrors.
    Pediatrics 2017 May 24. Epub 2017 May 24.
    Departments of Pediatrics and
    A 16-year-old boy with a recent diagnosis of night terrors was evaluated for recurrent early morning hypoglycemia after an early morning seizure. Evaluation in clinic with critical laboratories identified hyperinsulinemic hypoglycemia. Additional investigation revealed a sporadic insulinoma as the etiology of his hypoglycemia and all symptoms were resolved after pancreaticoduodenectomy. Read More

    Peptide Receptor Radionuclide Therapy with (177)Lu-DOTATATE for Metastatic Neuroendocrine Tumor Occurring in Association with Multiple Endocrine Neoplasia Type 1 and Cushing's Syndrome.
    World J Nucl Med 2017 Apr-Jun;16(2):126-132
    Radiation Medicine Centre, Bhabha Atomic Research Centre, Tata Memorial Centre Annexe, Parel, Mumbai, Maharashtra, India.
    Neuroendocrine tumor (NET) occurring in association with other endocrine syndromes forms a distinct entity. The aim was to assess the therapy response profile of the routine peptide receptor radionuclide therapy (PRRT) in this relatively uncommon but clinically challenging subgroup of patients. A retrospective analysis was undertaken from the case records from those who were treated with (177)Lu-DOTATATE for metastatic NET. Read More

    MEN1 in children and adolescents: Data from patients of a regional referral center for hereditary endocrine tumors.
    Endocrine 2017 May 22. Epub 2017 May 22.
    Department of Surgery and Translational Medicine, University of Florence, Largo Palagi 1, 50139, Florence, Italy.
    Purpose: To retrospectively evaluate the age of onset of MEN1-associated lesions in a group of affected children and adolescents and to compare the clinical features of our series with the evidence derived from the literature.

    Methods: The study population consisted of 22 Italian children and adolescents (age 6-31 years at the time of the inclusion in this study) all with a clinical and/or a genetic diagnosis of MEN1 performed before the age of 16 who have been followed-up regularly from 1998 to 2016 at the Regional Referral Center for Hereditary Endocrine Tumors. Clinical, biochemical, imaging and genetic data have been collected for each patient. Read More

    Epigenetic pathway inhibitors represent potential drugs for treating pancreatic and bronchial neuroendocrine tumors.
    Oncogenesis 2017 May 15;6(5):e332. Epub 2017 May 15.
    Academic Endocrine Unit, OCDEM, University of Oxford, Churchill Hospital, Headington, Oxford, UK.
    Cancer is associated with alterations in epigenetic mechanisms such as histone modifications and methylation of DNA, and inhibitors targeting epigenetic mechanisms represent a novel class of anti-cancer drugs. Neuroendocrine tumors (NETs) of the pancreas (PNETs) and bronchus (BNETs), which may have 5-year survivals of <50% and as low as 5%, respectively, represent targets for such drugs, as >40% of PNETs and ~35% of BNETs have mutations of the multiple endocrine neoplasia type 1 (MEN1) gene, which encodes menin that modifies histones by interacting with histone methyltransferases. We assessed 9 inhibitors of epigenetic pathways, for their effects on proliferation, by CellTiter Blue assay, and apoptosis, by CaspaseGlo assay, using 1 PNET and 2 BNET cell lines. Read More

    Germline and somatic genetic changes in multicentric tumors obtained from a patient with multiple endocrine neoplasia type 1.
    Hum Genome Var 2017 27;4:17013. Epub 2017 Apr 27.
    Shizuoka Cancer Center, Shizuoka, Japan.
    Multiple endocrine neoplasia type 1 (MEN1) is a hereditary cancer syndrome caused by germline mutations of the MEN1 gene located in chromosome 11q13. In patients with MEN1, multicentric tumors develop in the involved organs; however, precise evaluation of genetic changes in these multicentric tumors has not been performed. In the present study, using whole-exome sequencing, we analyzed germline and somatic genetic changes in blood cells, two pancreatic endocrine tumors and one duodenal tumor obtained from a patient with MEN1 gastrinoma. Read More

    Hibernoma and multiple endocrine neoplasia type 1 syndrome: A non-fortuitous association? A case report and literature review.
    Ann Endocrinol (Paris) 2017 May 4. Epub 2017 May 4.
    Department of endocrinology and diabetes, Lyon-Sud hospital, hospices civils de Lyon, 165, chemin du Grand-Revoyet, 69310 Pierre-Bénite, France. Electronic address:

    Genetics of Multiple Endocrine Neoplasia Type 1/Multiple Endocrine Neoplasia Type 2 Syndromes.
    Endocrinol Metab Clin North Am 2017 Jun 18;46(2):491-502. Epub 2017 Mar 18.
    Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, 1400 Pressler Street, Houston, TX 77030, USA. Electronic address:
    Multiple endocrine neoplasia syndromes types 1 and 2 represent well-characterized yet clinically heterogeneous hereditary conditions for which diagnostic and management recommendations exist; genetic testing for these inherited endocrinopathies is included in these guidelines and is an important part of identifying affected patients and their family members. Understanding of these mature syndromes is challenged as more individuals undergo genetic testing and genetic data are amassed, with the potential to create clinical conundrums that may have an impact on individualized approaches to management and counseling. Clinicians who diagnose and treat patients with MEN syndromes should be aware of these possibilities. Read More

    Management of a multiple endocrine neoplasia type 1 during pregnancy: A case report and review of the literature.
    J Exp Ther Oncol 2016 Jul;11(3):217-220
    Department of Perinatology, Zekai Tahir Burak Women's Health Education and Research Hospital, Ankara, Turkey.
    Multiple Endocrine Neoplasia Type 1 (MEN1) or Wermer's syndrome is a rare hereditary endocrine syndrome with high penetrance caused by mutations in MEN1 tumor suppressor gene. MEN1 is characterized by hyperplasia or tumoral enlargement in a number of endocrine organs (parathyroid glands, pancreas, pituitary gland, adrenal gland) and it could be hormonally active or inactive. MEN1 is a significant cause of morbidity due to hormone secretion and mass effect. Read More

    Phase II study of lanreotide autogel in Japanese patients with unresectable or metastatic well-differentiated neuroendocrine tumors.
    Invest New Drugs 2017 May 3. Epub 2017 May 3.
    Clinical Research Institute, National Hospital Organization Kyoto Medical Center, 1-1 Mukaihata-cho, Fukakusa, Fushimi-ku, Kyoto, Japan.
    Background Lanreotide is a long-acting somatostatin analog with demonstrated efficacy against enteropancreatic neuroendocrine tumor (NET) in the phase III (CLARINET) study. Materials and Methods In this single-arm study, Japanese patients with grade (G) 1/G2 NET received lanreotide (120 mg/4 weeks) for 48 weeks. Those who completed the study were enrolled in a long-term extension study. Read More

    Analysis of differentially expressed microRNAs in MEN1 parathyroid adenomas.
    Am J Transl Res 2017 15;9(4):1743-1753. Epub 2017 Apr 15.
    Unit of Bone and Mineral Metabolic Diseases, Department of Surgery and Translational Medicine, University of FlorenceFlorence, Italy.
    Multiple Endocrine Neoplasia type 1 (MEN1) syndrome is a rare complex tumor-predisposing hereditary disorder, inherited in an autosomal dominant manner (OMIM 131100). MEN1 is characterized by tumors of the parathyroids, the neuroendocrine cells of the gastro-entero-pancreatic tract, and the anterior pituitary. The molecular mechanisms that control parathyroid tumorigenesis are still poorly understood. Read More

    Hypersecretion of ACTH and PRL from pituitary adenoma in MEN1, adequately managed by medical therapy.
    Endocrinol Diabetes Metab Case Rep 2017 6;2017. Epub 2017 Apr 6.
    The 1st Department of Internal Medicine, Wakayama Medical University, WakayamaJapan.
    Summary: A 54-year-old man had gastrinoma, parathyroid hyperplasia and pituitary tumor. His family history indicated that he might have multiple endocrine neoplasia type 1 (MEN1). MEN1 gene analysis revealed a heterozygous germline mutation (Gly156Arg). Read More

    Thymoma (World Health Organization type B3) with neuroendocrine differentiation in multiple endocrine neoplasia type 1.
    J Surg Case Rep 2017 Apr 4;2017(4):rjx071. Epub 2017 Apr 4.
    Department of Cardiovascular Surgery, Faculty of Medicine, University of Miyazaki, Kiyotake, Miyazaki,Japan.
    Thymic epithelial tumors occur in 1-5% of patients with multiple endocrine neoplasia type 1 (MEN 1). Majority of these thymic epithelial tumors are thymic carcinoids and patients with thymoma in MEN 1 is rare. Furthermore, thymoma with neuroendocrine differentiation was also rarely reported. Read More

    Natural History of MEN1 GEP-NET: Single-Center Experience After a Long Follow-Up.
    World J Surg 2017 Apr 20. Epub 2017 Apr 20.
    Department of Surgery and Translational Medicine, University of Florence, Largo Brambilla 3, 50139, Florence, Italy.
    Background: The multiple endocrine neoplasia type 1 syndrome (MEN1) natural history is poorly evaluated, and few single-institution experiences about hereditary gastroenteropancreatic neuroendocrine tumors (GEP-NET) are reported. Our purpose is to analyze the role of GEP-NET in MEN1-related death, as well as the behavior of these lesions during follow-up.

    Methods: The study population consists of 77 patients diagnosed with MEN1 GEP-NET, regularly followed up since 1990. Read More

    A MEN1 pancreatic neuroendocrine tumour mouse model under temporal control.
    Endocr Connect 2017 May 18;6(4):232-242. Epub 2017 Apr 18.
    Academic Endocrine UnitOCDEM, University of Oxford, Churchill Hospital, Oxford, UK
    Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterised by occurrence of parathyroid tumours and neuroendocrine tumours (NETs) of the pancreatic islets and anterior pituitary. The MEN1 gene, encoding menin, is a tumour suppressor, but its precise role in initiating in vivo tumourigenesis remains to be elucidated. The availability of a temporally controlled conditional MEN1 mouse model would greatly facilitate the study of such early tumourigenic events, and overcome the limitations of other MEN1 knockout models, in which menin is lost from conception or tumour development occurs asynchronously. Read More

    Generation of non-integrated induced pluripotent stem cells from a 23-year-old male with multiple endocrine neoplasia type 1 syndrome.
    Stem Cell Res 2017 Jan 7;18:70-72. Epub 2016 Dec 7.
    Institute of Public Health, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, Guangzhou, China; South China Institute for Stem Cell Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, Guangzhou, China; Guangdong Provincial Key Laboratory of Biocomputing, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, Guangzhou, China. Electronic address:
    Urine resource cells were collected from a 23-year-old male with multiple endocrine neoplasia type 1 syndrome (MEN1) for generating iPS cells with episomal plasmids. Two stable iPSC lines with free of episomal plasmid were established. The patient has a heterozygous G>T mutation on the exon 9 of Men1 gene that was confirmed by sequencing analysis on all resulted cell lines. Read More

    Generation of non-integrated induced pluripotent stem cells from a 59-year-old female with multiple endocrine neoplasia type 1 syndrome.
    Stem Cell Res 2017 Jan 9;18:64-66. Epub 2016 Dec 9.
    Institute of Public Health, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, Guangzhou, China; South China Institute for Stem Cell Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, Guangzhou, China; Guangdong Provincial Key Laboratory of Biocomputing, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, Guangzhou, China. Electronic address:
    Urine resource cells were collected from a 59-year-old female patient with multiple endocrine neoplasia type 1 syndrome (MEN1) for generating iPS cells with episomal plasmids carrying Oct4, Sox2, Klf4 and miR-302-367. The patient sustained a heterozygous G>T transition mutation on the exon 9 of Men1 gene that was confirmed by sequencing analysis on the obtained iPSC lines. Karyotyping indicated the chromosomes with normal appearances and numbers. Read More

    Neuroendocrine Tumors in the Stomach, Duodenum, and Pancreas Accompanied by Novel MEN1 Gene Mutation.
    Korean J Gastroenterol 2017 Mar;69(3):181-186
    Division of Gastroenterology, Department of Internal Medicine, Presbyterian Medical Center, Jeonju, Korea.
    Multiple endocrine neoplasia type 1 (MEN1) syndrome is a relatively rare disease, characterized by the occurrence of multiple endocrine tumors in the parathyroid and pituitary glands as well as the pancreas. Here, we report a case of MEN1 with neuroendocrine tumors (NETs) in the stomach, duodenum, and pancreas. A 53-year-old man visited our hospital to manage gastric NET. Read More

    Utility of FDG-PET Imaging for Risk Stratification of Pancreatic Neuroendocrine Tumors in MEN1.
    J Clin Endocrinol Metab 2017 Jun;102(6):1926-1933
    Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart, Tasmania 7000, Australia.
    Context: Patients with multiple endocrine neoplasia type 1 (MEN1) are at high risk of malignant pancreatic neuroendocrine tumors (pNETs). Structural imaging is typically used to screen for pNETs but is suboptimal for stratifying malignant potential.

    Objective: To determine the utility of fluorodeoxyglucose (18F) positron emission tomography/computed tomography (18F-FDG PET/CT) for predicting the malignant potential of pNETs in MEN1. Read More

    MEN1-Dependent Breast Cancer: Indication for Early Screening? Results From the Dutch MEN1 Study Group.
    J Clin Endocrinol Metab 2017 Jun;102(6):2083-2090
    Department of Endocrine Oncology, University Medical Center Utrecht, 3584 CX Utrecht, The Netherlands.
    Objective: Multiple endocrine neoplasia type 1 (MEN1) is associated with an early-onset elevated breast cancer risk. This finding potentially has implications for breast cancer screening for women with MEN1, and therefore it is necessary to assess whether other risk factors are involved to identify those at greatest risk.

    Design: A cross-sectional case control study was performed using the Dutch MEN1 cohort, including >90% of the adult Dutch MEN1 population. Read More

    Radiological surveillance in multiple endocrine neoplasia type 1: a double-edged sword?
    Endocr Connect 2017 Apr 15;6(3):151-158. Epub 2017 Mar 15.
    Wolfson Diabetes and Endocrine ClinicInstitute of Metabolic Science, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
    Context: Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition characterised by the predisposition to hyperplasia/tumours of endocrine glands. MEN1-related disease, moreover, malignancy related to MEN1, is increasingly responsible for death in up to two-thirds of patients. Although patients undergo radiological and biochemical surveillance, current recommendations for radiological monitoring are based on non-prospective data with little consensus or evidence demonstrating improved outcome from this approach. Read More

    Association between neuroendocrine tumors biomarkers and primary tumor site and disease type based on total (68)Ga-DOTATATE-Avid tumor volume measurements.
    Eur J Endocrinol 2017 May;176(5):575-582
    Endocrine Oncology BranchNational Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
    Objective: To determine the association between neuroendocrine tumor (NET) biomarker levels and the extent of disease as assessed by (68)Ga DOTATATE PET/CT imaging.

    Design: A retrospective analysis of a prospective database of patients with NETs.

    Methods: Fasting plasma chromogranin A (CgA), neuron-specific enolase (NSE), gastrin, glucagon, vasoactive intestinal peptide (VIP) and pancreatic polypeptide (PP), and 24-h urinary 5-hydroxyindoleacetic acid (5-HIAA) levels were measured. Read More

    Enhancer-Mediated Oncogenic Function of the Menin Tumor Suppressor in Breast Cancer.
    Cell Rep 2017 Mar;18(10):2359-2372
    Department of Medical Oncology, Dana-Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Boston 02215, MA, USA; Center for Functional Cancer Epigenetics, Dana-Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Boston 02215, MA, USA. Electronic address:
    While the multiple endocrine neoplasia type 1 (MEN1) gene functions as a tumor suppressor in a variety of cancer types, we explored its oncogenic role in breast tumorigenesis. The MEN1 gene product menin is involved in H3K4 trimethylation and co-activates transcription. We integrated ChIP-seq and RNA-seq data to identify menin target genes. Read More

    Is estrogen exposure a protective factor for pancreatic neuroendocrine tumours in female patients with multiple endocrine neoplasia syndrome type 1?
    Clin Endocrinol (Oxf) 2017 Jun 6;86(6):791-797. Epub 2017 Apr 6.
    Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
    Objective: Pancreatic neuroendocrine tumours (PNETs) are the most common cause of death in patients with multiple endocrine neoplasia type 1 (MEN1). Women have been shown to have improved survival, which may suggest a possible protective effect of female sex hormones. The aim of this study was to evaluate the relationship between estrogen exposure and PNET tumourigenesis, tumour growth and survival in female MEN1 patients with these tumours. Read More

    Menin and PRMT5 suppress GLP1 receptor transcript and PKA-mediated phosphorylation of FOXO1 and CREB.
    Am J Physiol Endocrinol Metab 2017 Mar 7:ajpendo.00241.2016. Epub 2017 Mar 7.
    University of Pennsylvania
    Menin is a scaffold protein that interacts with several epigenetic mediators to regulate gene transcription, and suppresses pancreatic beta cell proliferation. Tamoxifen inducible deletion of multiple endocrine neoplasia type 1 (MEN1) gene, which encodes the protein menin, increases beta cell mass in multiple murine models of diabetes and ameliorates diabetes. Glucagon-like-peptide-1 (GLP1) is another key physiological modulator of beta cell mass and glucose homeostasis. Read More

    A case of type 1 multiple endocrine neoplasia with esophageal stricture successfully treated with endoscopic balloon dilation and local steroid injection combined with surgical resection of gastrinomas.
    BMC Gastroenterol 2017 Mar 7;17(1):37. Epub 2017 Mar 7.
    Division of Endoscopy, Shizuoka Cancer Center, 1007, Shimonagakubo, Nagaizumi, Suntogun, Shizuoka, 411-8777, Japan.
    Background: In type 1 multiple endocrine neoplasia (MEN1), esophageal diseases association with excessive gastrin secretion in Zollinger-Ellison syndrome (ZES) sometimes develop. Here, we reported a case of MEN1/ZES, who developed dysphagia due to reflux esophagitis with severe esophageal stricture. Treatment for his esophageal stricture and ZES was discussed. Read More

    The Wandering Mesenteric Lymph Node: Delayed 68Ga-DOTANOC PET/CT Imaging to Overcome a Potential Pitfall.
    Clin Nucl Med 2017 May;42(5):e253-e254
    From the *Nuclear Medicine, and †Radiology, Sant'Orsola University Hospital, Bologna, Italy.
    A patient multiple endocrine neoplasia type 1 presenting with radiological suspicion of pancreatic neuroendocrine tumor relapse after surgical and somatostatin analog treatment underwent restaging Ga-DOTANOC PET/CT. Standard and delayed images detected an area of focal intense uptake moving from the left para-aortic to the paracaval region. The lesion was identified at previous imaging in different abdominal locations (eg, adjacent to the duodenal wall at presurgical PET and in the aortocaval region at restaging contrast-enhanced CT). Read More

    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 & Groupe d'Etude des Tumeurs Endocrines.
    Ann Surg 2017 Mar 15. Epub 2017 Mar 15.
    *Department of Thoracic and Endocrine Surgery, University Hospitals of Geneva and Faculty of Medicine of Geneva, Geneva, Switzerland †Department of General and Endocrine Surgery, University Hospital of Lille, Lille, France ‡Department of Endocrinology, University Hospital of Lille, Lille, France §Department of Digestive and Endocrine Surgery, University Hospital of Nantes, Nantes, France ¶Department of Endocrinology, University Hospital of Nantes, Nantes, France ||Department of Endocrine Surgery, La Conception University Hospital, Marseille, France **Institut Paoli-Calmettes, Marseille, France ††Department of Digestive and Endocrine Surgery, University Hospital of Reims, Reims, France ‡‡Department of Hepato-Gastroenterology and Digestive Oncology, University Hospital of Reims, Reims, France §§Department of Surgery, University Hospital of Lyon, Lyon, France ¶¶Department of Digestive and Endocrine Surgery, University Hospital of Lyon Sud and 2/EA 7425 HESPER, Health Services and Performance Research, University Claude Bernard Lyon 1, Lyon, France ||||Department of Endocrinology, Hospital Louis Pradel, University Lyon I, Lyon, France ***Department of Thoracic, Vascular and Endocrine Surgery, University Hospital of Grenoble, Grenoble, France †††Department of Endocrinology and Diabetology, University Hospital of Grenoble, Grenoble, France ‡‡‡Department of Digestive and Endocrine Surgery, University Hospital La Pitié Salpétrière, Paris, France §§§Department of Nuclear Medicine and Endocrine Oncology, Gustave Roussy, University of Paris Sud, Villejuif, France ¶¶¶Department of Gastroenterology and Pancreatology, Beaujon Hospital, University Paris 7, Clichy, France ||||||Department of Endocrinology, General Hospital of Chambéry, Chambéry, France ****Department of Endocrine Surgery, University Hospital of Dijon, and INSERM, U866, Epidemiology and Clinical Research in Digestive Oncology Team, and INSERM, CIC1432, Clinical Epidemiology Unit, University Hospital of Dijon, Clinical Investigation Center, Clinical Epidemiology/Clinical Trials Unit, Dijon, France.
    Objective: 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. Read More

    Management of MEN1 Related Nonfunctioning Pancreatic NETs: A Shifting Paradigm: Results From the DutchMEN1 Study Group.
    Ann Surg 2017 Mar 2. Epub 2017 Mar 2.
    *Department of Endocrine Surgical Oncology and Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands †Imaging Division, University Medical Center Utrecht, Utrecht, The Netherlands ‡Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands §Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands ¶Department of Endocrinology, Radboud University Medical Center, Nijmegen, The Netherlands ||Departments of Endocrinology and Metabolism and Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands **Department of Endocrinology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands ††Department of Internal Medicine, Section of Endocrinology, VU University Medical Center, Amsterdam, The Netherlands ‡‡Department of Endocrinology and Metabolism, Academic Medical Center, Amsterdam, The Netherlands §§Department of Internal Medicine, Division of Endocrinology, Maastricht University Medical Center, Maastricht, The Netherlands ¶¶Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.
    Objective: To assess if surgery for Multiple Endocrine Neoplasia type 1 (MEN1) related nonfunctioning pancreatic neuroendocrine tumors (NF-pNETs) is effective for improving overall survival and preventing liver metastasis.

    Background: MEN1 leads to multiple early-onset NF-pNETs. The evidence base for guiding the difficult decision who and when to operate is meager. Read More

    Genetic confirmation that ependymoma can arise as part of multiple endocrine neoplasia type 1 (MEN1) syndrome.
    Acta Neuropathol 2017 Apr 25;133(4):661-663. Epub 2017 Feb 25.
    Division of Neuropathology, Department of Pathology, University of California, San Francisco, 513 Parnassus Ave, Health Sciences West 451, San Francisco, CA, 94143, USA.

    PROGNOSTIC FACTORS FOR SURVIVAL OF MEN1 PATIENTS WITH DUODENOPANCREATIC TUMORS METASTATIC TO THE LIVER: RESULTS FROM THE DMSG.
    Endocr Pract 2017 Jun 22;23(6):641-648. Epub 2017 Feb 22.
    Objective: Duodenopancreatic neuroendocrine tumors (DP-NETs) develop in a majority of patients with multiple endocrine neoplasia type 1 (MEN1) and are the leading cause of death. Overall survival (OS) and prognostic factors for patients with liver metastases from DP-NETs are not known.

    Methods: This was a cohort study using the Dutch National MEN1 database, which includes >90% of the Dutch MEN1 population treated between 1990 and 2014. Read More

    B-mode and contrast-enhancement characteristics of small nonincidental neuroendocrine pancreatic tumors.
    Endosc Ultrasound 2017 Jan-Feb;6(1):49-54
    Medical Department, Caritas-Krankenhaus, Uhlandstr. 7 D-97980 Bad Mergentheim, Germany; Sino-German Research Center of Ultrasound in Medicine, The first Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
    Background And Objectives: Imaging of the pancreas for detection of neuroendocrine tumors is indicated as surveillance in multiple endocrine neoplasia type 1 (MEN1) or if typical clinical symptoms combined with hormone production raise the suspicion of a neuroendocrine tumor. Endoscopic ultrasound (EUS) is considered the best imaging modality to detect small pancreatic tumors. However, little is known about how small pancreatic neuroendocrine tumors (pNETs) present on EUS. Read More

    Foxa2, a novel protein partner of the tumour suppressor menin, is deregulated in mouse and human MEN1 glucagonomas.
    J Pathol 2017 May 27;242(1):90-101. Epub 2017 Mar 27.
    INSERM U1052, Lyon, France.
    Foxa2, known as one of the pioneer factors, plays a crucial role in islet development and endocrine functions. Its expression and biological functions are regulated by various factors, including, in particular, insulin and glucagon. However, its expression and biological role in adult pancreatic α-cells remain elusive. Read More

    Complicated Case Presentation: Management of Pancreatic Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1.
    Pancreas 2017 Mar;46(3):416-426
    From the *Department of Internal Medicine, †Division of Hematology/Oncology, Department of Medicine, ‡Division of Endocrinology and Metabolism, Department of Medicine, and §Section of Surgical Oncology/Hepatopancreaticobiliary Surgery, Division of General Surgery, Department of Surgery, University of California, San Francisco, San Francisco, CA.
    Multiple endocrine neoplasia type 1 (MEN1) is an inherited predisposition to tumors of the parathyroid glands, anterior pituitary, and pancreatic islet cells. In this review, we discuss the clinical case of a 45-year-old woman with MEN1 that was presented at the 2015 North American Neuroendocrine Tumor Society Symposium. In our review of this patient's complicated clinical course and subsequent operative management, we highlight controversies in the diagnosis and management of pancreatic neuroendocrine tumors in MEN1. Read More

    Genetics of multiple endocrine neoplasia type 1 syndrome: what's new and what's old.
    F1000Res 2017 24;6. Epub 2017 Jan 24.
    EndOsMet Unit, Villa Donatello, Piazzale Donatello 2, Florence 50100, Italy; Hercolani Clinical Center, Via D'Azeglio 46, Bologna 40136, Italy.
    Despite its identification in 1997, the functions of the MEN1 gene-the main gene underlying multiple endocrine neoplasia type 1 syndrome-are not yet fully understood. In addition, unlike the RET-MEN2 causative gene-no hot-spot mutational areas or genotype-phenotype correlations have been identified. More than 1,300 MEN1 gene mutations have been reported and are mostly "private" (family specific). Read More

    Limited Value of Ga-68-DOTATOC-PET-CT in Routine Screening of Patients with Multiple Endocrine Neoplasia Type 1.
    World J Surg 2017 Jun;41(6):1521-1527
    Department of Visceral, Thoracic and Vascular Surgery, University Hospital of Giessen and Marburg, Philipps University of Marburg, Marburg, Germany.
    Background: Routine screening is recommended for patients with multiple endocrine neoplasia type 1 (MEN1) to enable early detection and treatment of associated neuroendocrine neoplasms (NEN). Gallium(68)-DOTATOC-Positron emission tomography combined with computed tomography (Ga-68-DOTATOC-PET-CT) is a very sensitive and specific imaging technique for the detection of sporadic neuroendocrine tumors. The present study evaluated the value of Ga-68-DOTATOC-PET-CT in routine screening of patients with MEN1. Read More

    Type 2 gastric neuroendocrine tumor: report of one case.
    Transl Gastroenterol Hepatol 2016 30;1:88. Epub 2016 Nov 30.
    Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing 100029, China.
    In this article we reported a female patient with type 2 gastric neuroendocrine tumor (NET). The patient developed upper abdominal pain, acid reflux, heartburn, nausea, and vomiting without obvious cause 16 years ago. Later, a tumor was found in her stomach. Read More

    Metastatic multiple endocrine neoplasia type 1: report of one case.
    Transl Gastroenterol Hepatol 2016 23;1:73. Epub 2016 Sep 23.
    Department of Pancreatic Surgery, Zhongshan Hospital, Fudan University, Shanghai 200032, China.
    A 46-year-old Chinese woman was admitted to our hospital because of presence of space-occupying lesions in the liver for 2 months in April, 2015. She had a family history of multiple endocrine neoplasia type 1 (MEN1) and physical examination is unremarkable. Previously, she has performed surgery for primary pituitary tumor in 2002 and radiosurgery for its recurrence. Read More

    [Multiple Endocrine Neoplasia Type 1(MEN1)Presenting with Hypoglycemic Attacks - A Case Report].
    Gan To Kagaku Ryoho 2016 Nov;43(12):2196-2198
    Dept. of Surgery, Kobe Ekisaikai Hospital.
    A woman in her 60s visited our hospital because of frequent hypoglycemia and episodes of unconsciousness over the last 6 years. A 4 cm tumor was detected on the pancreatic tail using abdominal computed tomography and ultrasonography. An insulinoma was strongly suspected from the results of the fasting test and glucagon load test, and a distal pancreatectomy with splenectomy was performed. Read More

    Multiple endocrine neoplasia syndrome type 1: institution, management, and data analysis of a nationwide multicenter patient database.
    Endocrine 2017 Jan 28. Epub 2017 Jan 28.
    Department of Surgery and Translational Medicine, University of Florence, Largo Palagi 1, 50139, Florence, Italy.
    Objective: The aim of this study was to integrate European epidemiological data on patients with multiple endocrine neoplasia type 1 by creating an Italian registry of this syndrome, including clinical and genetic characteristics and therapeutic management.

    Methods: Clinical, familial and genetic data of patients with multiple endocrine neoplasia type 1, diagnosed, treated, and followed-up for a mean time of 11.3 years, in 14 Italian referral endocrinological centers, were collected, over a 3-year course (2011-2013), to build a national electronic database. Read More

    Loss of menin in osteoblast lineage affects osteocyte-osteoclast crosstalk causing osteoporosis.
    Cell Death Differ 2017 Apr 20;24(4):672-682. Epub 2017 Jan 20.
    Institute of Comparative Molecular Endocrinology, University of Ulm, Ulm D-89081, Germany.
    During osteoporosis bone formation by osteoblasts is reduced and/or bone resorption by osteoclasts is enhanced. Currently, only a few factors have been identified in the regulation of bone integrity by osteoblast-derived osteocytes. In this study, we show that specific disruption of menin, encoded by multiple endocrine neoplasia type 1 (Men1), in osteoblasts and osteocytes caused osteoporosis despite the preservation of osteoblast differentiation and the bone formation rate. Read More

    Lung adenocarcinoma and adrenocortical carcinoma in a patient with multiple endocrine neoplasia type 1.
    Respir Med Case Rep 2017 18;20:77-81. Epub 2016 Dec 18.
    Department of Internal Medicine, Ojiya General Hospital, Niigata, Japan; Center of Diabetes, Endocrinology and Metabolism, Joetsu General Hospital, Niigata, Japan.
    Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder caused by heterozygous germline mutations in the tumor suppressor gene MEN1, which encodes a nuclear protein, menin. MEN1 is characterized by the combined occurrence of tumors involving the pituitary gland, pancreatic islets, and parathyroid glands. Additionally, patients with MEN1 often exhibit adrenal tumors. Read More

    Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process.
    Am J Med 2017 May 21;130(5):603-605. Epub 2016 Dec 21.
    Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, Mayo Clinic, Rochester, Minn. Electronic address:
    Background: Zollinger-Ellison syndrome is a rare cause of tumoral hypergastrinemia; 1 of 5 patients with this syndrome also has multiple endocrine neoplasia type 1. The diagnosis of this disease is complicated by the widespread use of proton pump inhibitors that can elevate serum gastrin levels, the cornerstone for biochemical diagnosis. Abrupt discontinuation of proton pump inhibitors could lead to adverse outcomes. Read More

    Upregulation of RPA2 promotes NF-κB activation in breast cancer by relieving the antagonistic function of menin on NF-κB-regulated transcription.
    Carcinogenesis 2017 Feb;38(2):196-206
    Department of Nutrition, College of Medicine and Nursing, Hung Kuang University, Taichung 43302, Taiwan.
    RPA2, a subunit of the heterotrimeric replication protein A (RPA) complex, is overexpressed in various cancers. In this study, we showed a significant RPA2 upregulation in breast cancer tissues and cell lines. Ectopic expression of RPA2 in MCF7 and MDA-MB-231 cells promoted cell proliferation, adhesion, migration and invasion, and induced epithelial-mesenchymal transition (EMT) of MCF7 cells. Read More

    1 OF 46