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Xiao-Hua Jiang Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Jie-Li Lu Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Bin Cui Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China
Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Yong-Ju Zhao Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Wei-qing Wang Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Jian-Min Liu Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Wen-Qiang Fang Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Ya-Nan Cao Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Yan Ge Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Chang-xian Zhang Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Huguette Casse Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Xiao-Ying Li Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China
Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Guang Ning Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China
Shanghai Clinical Center for Endocrine and Metabolic Diseases,, Laboratory for Endocrine and Metabolic Diseases,, Department of Radiological Medicine,, Laboratoire Genetique et Cancer,, Shanghai Key Laboratory for Endocrine Tumours,, Shanghai Institute of Endocrinology and Metabolism and Chinese-French Laboratory of Genomics and Life Sciences, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, 197 Ruijin 2nd Road, Shanghai 200025, China

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Introduction Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant inherited disorder characterized by frequent occurrence of tumours of parathyroid, pancreatic islet and anterior pituitary, sometimes in combination with other rare

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A A Pannett Nuffield Department of Medicine, John Radcliffe Hospital, Headington, Oxford, UK.

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R V Thakker Nuffield Department of Medicine, John Radcliffe Hospital, Headington, Oxford, UK.

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Combined clinical and laboratory investigations of multiple endocrine neoplasia type 1 (MEN1) have resulted in an increased understanding of this disorder which may be inherited as an autosomal dominant condition. Defining the features of each disease manifestation in MEN1 has improved patient management and treatment, and has also facilitated a screening protocol to be instituted. The application of the techniques of molecular biology has enabled the identification of the gene causing MEN1 and the detection of mutations in patients. The function of the protein encoded by the MEN1 gene has been shown to be in the regulation of JunD-mediated transcription but much still remains to be elucidated. However, these recent advances provide for the identification of mutant MEN1 gene carriers who are at a high risk of developing this disorder and thus require regular and biochemical screening to detect the development of endocrine tumours.

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C R C Pieterman Division of Internal Medicine and Dermatology, Division of Biomedical Genetics, Division of Surgical Specialties, Department of Internal Medicine, University Medical Center Utrecht, Internal post number L.00.408, PO Box 85500, 3508 GA Utrecht, The Netherlands

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E B Conemans Division of Internal Medicine and Dermatology, Division of Biomedical Genetics, Division of Surgical Specialties, Department of Internal Medicine, University Medical Center Utrecht, Internal post number L.00.408, PO Box 85500, 3508 GA Utrecht, The Netherlands
Division of Internal Medicine and Dermatology, Division of Biomedical Genetics, Division of Surgical Specialties, Department of Internal Medicine, University Medical Center Utrecht, Internal post number L.00.408, PO Box 85500, 3508 GA Utrecht, The Netherlands

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K M A Dreijerink Division of Internal Medicine and Dermatology, Division of Biomedical Genetics, Division of Surgical Specialties, Department of Internal Medicine, University Medical Center Utrecht, Internal post number L.00.408, PO Box 85500, 3508 GA Utrecht, The Netherlands

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J M de Laat Division of Internal Medicine and Dermatology, Division of Biomedical Genetics, Division of Surgical Specialties, Department of Internal Medicine, University Medical Center Utrecht, Internal post number L.00.408, PO Box 85500, 3508 GA Utrecht, The Netherlands

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H Th M Timmers Division of Internal Medicine and Dermatology, Division of Biomedical Genetics, Division of Surgical Specialties, Department of Internal Medicine, University Medical Center Utrecht, Internal post number L.00.408, PO Box 85500, 3508 GA Utrecht, The Netherlands

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M R Vriens Division of Internal Medicine and Dermatology, Division of Biomedical Genetics, Division of Surgical Specialties, Department of Internal Medicine, University Medical Center Utrecht, Internal post number L.00.408, PO Box 85500, 3508 GA Utrecht, The Netherlands

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G D Valk Division of Internal Medicine and Dermatology, Division of Biomedical Genetics, Division of Surgical Specialties, Department of Internal Medicine, University Medical Center Utrecht, Internal post number L.00.408, PO Box 85500, 3508 GA Utrecht, The Netherlands

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Introduction Thoracic and duodenopancreatic neuroendocrine tumors (dpNETs) can occur either sporadically or as a manifestation of an inherited syndrome, most importantly the multiple endocrine neoplasia type 1 (MEN1) syndrome. This is an autosomal

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Brian Harding
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Manuel C Lemos
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Anita A C Reed
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Gerard V Walls
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Jeshmi Jeyabalan
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Michael R Bowl
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Hilda Tateossian Academic Endocrine Unit, Mammalian Genetics Unit and Mary Lyon Centre, Department of Neuropathology, Unit of Clinical Biochemistry, Nuffield Department of Clinical Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism (OCDEM), Churchill Hospital, University of Oxford, Headington, Oxford OX3 7LJ, UK

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Nicky Sullivan Academic Endocrine Unit, Mammalian Genetics Unit and Mary Lyon Centre, Department of Neuropathology, Unit of Clinical Biochemistry, Nuffield Department of Clinical Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism (OCDEM), Churchill Hospital, University of Oxford, Headington, Oxford OX3 7LJ, UK

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Tertius Hough Academic Endocrine Unit, Mammalian Genetics Unit and Mary Lyon Centre, Department of Neuropathology, Unit of Clinical Biochemistry, Nuffield Department of Clinical Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism (OCDEM), Churchill Hospital, University of Oxford, Headington, Oxford OX3 7LJ, UK

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William D Fraser Academic Endocrine Unit, Mammalian Genetics Unit and Mary Lyon Centre, Department of Neuropathology, Unit of Clinical Biochemistry, Nuffield Department of Clinical Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism (OCDEM), Churchill Hospital, University of Oxford, Headington, Oxford OX3 7LJ, UK

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Olaf Ansorge Academic Endocrine Unit, Mammalian Genetics Unit and Mary Lyon Centre, Department of Neuropathology, Unit of Clinical Biochemistry, Nuffield Department of Clinical Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism (OCDEM), Churchill Hospital, University of Oxford, Headington, Oxford OX3 7LJ, UK

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Michael T Cheeseman Academic Endocrine Unit, Mammalian Genetics Unit and Mary Lyon Centre, Department of Neuropathology, Unit of Clinical Biochemistry, Nuffield Department of Clinical Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism (OCDEM), Churchill Hospital, University of Oxford, Headington, Oxford OX3 7LJ, UK

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Rajesh V Thakker
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Introduction Multiple endocrine neoplasia type 1 (MEN1), in man, is characterised by the combined occurrence of tumours of parathyroids, pancreatic islets and anterior pituitary ( Marx 2005 , Thakker 2006 ). Some patients may also develop adrenal

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P H Kann
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E Balakina
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D Ivan
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D K Bartsch
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S Meyer
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K-J Klose
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Th Behr
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P Langer
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T, Celik I, Rothmund M & Nies C 2000 Pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1: surgery or surveillance? Surgery 128 958 –966. Bolondi L , Li Bassi S, Gaiani S, Campione O, Marrano

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Nader Hussein Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France
Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France

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JieLi Lu Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France
Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France

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Huguette Casse Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France
Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France

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Sandra Fontanière Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France
Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France

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Anne-Marie Morera Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France

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Séverine Mazaud Guittot Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France

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Alain Calender Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France
Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France
Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France

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Nathalie Di Clemente Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France

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Chang X Zhang Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France
Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France
Université Claude Bernard Lyon, CNRS UMR5201, INSERM-INRA U418, INSERM, Groupe d'Etude Recherche, Lyon, France; Faculté de Médecine, Université Lyon 1, Lyon F-69003, France; Laboratoire Génétique Moléculaire, Signalisation et Cancer, 8 Avenue Rockefeller, Lyon F-69373, France

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Introduction The patients with multiple endocrine neoplasia type 1 (MEN1), a hereditary syndrome transmitted with an autosomal dominant trait, predispose to the occurrence of multiple endocrine tumours of the parathyroids, pancreas, anterior

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Elizabeth Grubbs Departments of Surgical Oncology, GI Medical Oncology, and Endocrine Neoplasia and Hormonal Disorders, University of Texas MD Anderson Cancer Center, Houston, Texas

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Daniel Halperin Departments of Surgical Oncology, GI Medical Oncology, and Endocrine Neoplasia and Hormonal Disorders, University of Texas MD Anderson Cancer Center, Houston, Texas

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Steven G Waguespack Departments of Surgical Oncology, GI Medical Oncology, and Endocrine Neoplasia and Hormonal Disorders, University of Texas MD Anderson Cancer Center, Houston, Texas

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Robert F Gagel Departments of Surgical Oncology, GI Medical Oncology, and Endocrine Neoplasia and Hormonal Disorders, University of Texas MD Anderson Cancer Center, Houston, Texas

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Overview The multiple endocrine neoplasia syndromes are a varied group of disorders that include multiple endocrine neoplasia types 1 and 2 (MEN 1, MEN 2), von Hippel-Lindau (VHL) disease , Carney complex (CC), hereditary pheochromocytoma

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Yulong Li Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases

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William F Simonds National Institutes of Health, Bethesda, Maryland, USA

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, multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2A (MEN2A), and the hyperparathyroidism-jaw tumor syndrome (HPT-JT) are familial syndromes of hyperparathyroidism that are associated with a spectrum of endocrine tumors and other

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Francesca Marini Fondazione FIRMO Onlus, Italian Foundation for the Research on Bone Diseases, Florence, Italy

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Francesca Giusti Donatello Bone Clinic, Villa Donatello Hospital, Sesto Fiorentino, Italy

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Maria Luisa Brandi Fondazione FIRMO Onlus, Italian Foundation for the Research on Bone Diseases, Florence, Italy
Donatello Bone Clinic, Villa Donatello Hospital, Sesto Fiorentino, Italy

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in the context of inherited tumor syndromes ( Ishida & Lam 2022 ), such as multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 4 (MEN4), tuberous sclerosis complex (TSC), neurofibromatosis type 1 (NF1), and von Hippel

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Jerena Manoharan Department of Visceral Thoracic and Vascular Surgery, Philipps University Marburg, Marburg, Germany

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Max B Albers Department of Visceral Thoracic and Vascular Surgery, Philipps University Marburg, Marburg, Germany

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Detlef K Bartsch Department of Visceral Thoracic and Vascular Surgery, Philipps University Marburg, Marburg, Germany

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of Ga-68-DOTATOC-PET-CT in routine screening of patients with multiple endocrine neoplasia Type 1 . World Journal of Surgery 41 1521 – 1527 . ( doi:10.1007/s00268-017-3907-9 ) Anlauf M Garbrecht N Henopp T Schmitt A Schlenger

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