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Iuri Martin Goemann Thyroid Unit, Endocrine Division, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil

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Vicente Rodrigues Marczyk Thyroid Unit, Endocrine Division, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil

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Mirian Romitti Institut de Recherche Interdisciplinaire en Biologie Humaine et Moléculaire, Université Libre de Bruxelles, Brussels, Belgium

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Simone Magagnin Wajner Thyroid Unit, Endocrine Division, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil

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Ana Luiza Maia Thyroid Unit, Endocrine Division, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil

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several pathways, particularly in the context of neoplasia. This broad issue has been recently reviewed by our group and others and will not be discussed here ( Davis et al. 2016 , Goemann et al. 2017 ). It should also be noted that rT 3 , which is

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-18-0097
Volume/Issue:
Volume 25: Issue 12
Free access
Joanna Grey Association for Multiple Endocrine Neoplasia Disorders (AMEND), Tunbridge Wells, Kent, UK

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Kym Winter Association for Multiple Endocrine Neoplasia Disorders (AMEND), Tunbridge Wells, Kent, UK

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Introduction Multiple endocrine neoplasia type 2 (MEN2) refers collectively to 2 main distinct types of autosomal dominantly inherited neuroendocrine tumour (NET) syndromes ( Barakat et al. 2004 ); MEN type 2A and MEN type 2B (also known as

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-17-0335
Volume/Issue:
Volume 25: Issue 2
Free access
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

Article Type:
Editorial
DOI:
https://doi.org/10.1530/ERC-20-0201
Volume/Issue:
Volume 27: Issue 8
Free access
Iuri Martin Goemann Thyroid Section, Endocrine Division, Hospital de Clínicas de Porto Alegre

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Mirian Romitti Thyroid Section, Endocrine Division, Hospital de Clínicas de Porto Alegre

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Erika L Souza Meyer Department of Internal Medicine, Universidade Federal de Ciências da Saúde de Porto Alegre (UFCSPA), Porto Alegre, Rio Grande do Sul, Brazil

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Simone Magagnin Wajner Thyroid Section, Endocrine Division, Hospital de Clínicas de Porto Alegre

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Ana Luiza Maia Thyroid Section, Endocrine Division, Hospital de Clínicas de Porto Alegre

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proliferation and differentiation ( Kress et al . 2009 b , Dentice et al . 2013 ). Indeed, changes in the expression levels of deiodinases are present in several malignant human neoplasias. DIO1 downregulation occurs in renal, lung, hepatic and prostate

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-17-0192
Volume/Issue:
Volume 24: Issue 11
Free access
Thomas J Giordano Divisions of Anatomic Pathology and Molecular & Genomic Pathology, Departments of Pathology and Internal Medicine, Michigan Medicine, University of Michigan, Ann Arbor, Michigan, USA

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) that can predict response to immunotherapy ( Goodman et al . 2017 , Yarchoan et al . 2017 , Steuer & Ramalingam 2018 ), it may be especially relevant and revealing in endocrine neoplasia ( Hellmann et al . 2018 , Kim et al . 2018 a ). Many

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-18-0116
Volume/Issue:
Volume 25: Issue 8
Free access
Elizabeth G Grubbs Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Ronald M Lechan Department of Medicine, Tufts University School of Medicine, Boston, Massachusetts, USA

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Beth Edeiken-Monroe Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Gilbert J Cote Department of Endocrine Neoplasia and Hormonal Disorders, University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Chardria Trotter Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Arthur S Tischler Department of Pathology, Tufts University School of Medicine, Boston, Massachusetts, USA

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Robert F Gagel Department of Pathology, Tufts University School of Medicine, Boston, Massachusetts, USA

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physician he saw very quickly pieced together a history consistent with a recently identified syndrome, multiple endocrine neoplasia type 2A – the association of medullary thyroid carcinoma (MTC), hyperparathyroidism and pheochromocytoma ( Steiner et al

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-20-0108
Volume/Issue:
Volume 27: Issue 8
Free access
Rehannah Borup Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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Maria Rossing Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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Ricardo Henao Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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Yohei Yamamoto Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet
Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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Annelise Krogdahl Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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Christian Godballe Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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Ole Winther Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet
Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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Katalin Kiss Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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Lise Christensen Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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Estrid Høgdall Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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Finn Bennedbæk Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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Finn Cilius Nielsen Departments of, Clinical Biochemistry, Endocrinology and Metabolism, Pathology, Herlev Hospital, Pathology, Bispebjerg Hospital, University of Copenhagen, Pathology, ENT Head and Neck Surgery, Odense University Hospital, Bioinformatics Centre, University of Copenhagen, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, Danish Technical University, Informatics, Rigshospitalet

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and/or vascular invasion. Moreover, it is difficult to distinguish benign follicular adenoma (FA) from carcinoma. The road to follicular neoplasia is not completely understood. In contrast to the well-defined RET and BRAF mutations found in

Article Type:
Research Article
DOI:
https://doi.org/10.1677/ERC-09-0288
Volume/Issue:
Volume 17: Issue 3
Free access
Florian Bösch Department of General, Visceral, and Transplant Surgery, Ludwig-Maximilians-University Munich, Munich, Germany
Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany

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Katharina Brüwer Department of General, Visceral, and Transplant Surgery, Ludwig-Maximilians-University Munich, Munich, Germany
Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany

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Annelore Altendorf-Hofmann Department of General, Visceral und Vascular Surgery, Friedrich-Schiller University, Jena, Germany

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Christoph J Auernhammer Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany
Department of Internal Medicine 4, Ludwig-Maximilians-University Munich, Munich, Germany

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Christine Spitzweg Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany
Department of Internal Medicine 4, Ludwig-Maximilians-University Munich, Munich, Germany

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C Benedikt Westphalen Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany
Department of Medicine 3 and Comprehensive Cancer Center, Ludwig-Maximilians-University Munich, Munich, Germany

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Stefan Boeck Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany
Department of Medicine 3 and Comprehensive Cancer Center, Ludwig-Maximilians-University Munich, Munich, Germany

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Gabriele Schubert-Fritschle Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany
Munich Cancer Registry (MCR) of the Munich Tumour Centre (TZM), Institute for Medical Information Processing, Biometry, and Epidemiology (IBE), Ludwig-Maximilians-University Munich, Munich, Germany

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Jens Werner Department of General, Visceral, and Transplant Surgery, Ludwig-Maximilians-University Munich, Munich, Germany
Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany

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Volker Heinemann Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany
Department of Medicine 3 and Comprehensive Cancer Center, Ludwig-Maximilians-University Munich, Munich, Germany

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Thomas Kirchner Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany
Institute of Pathology, Ludwig-Maximilians-University, Munich, Germany

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Martin Angele Department of General, Visceral, and Transplant Surgery, Ludwig-Maximilians-University Munich, Munich, Germany
Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany

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Thomas Knösel Interdisciplinary Center of Neuroendocrine Tumors of the GastroEnteroPancreatic System, Ludwig-Maximilians-University of Munich, Munich, Germany
Institute of Pathology, Ludwig-Maximilians-University, Munich, Germany

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Introduction Neuroendocrine neoplasia (NENs) are a heterogeneous group of tumors, which can arise from neuroendocrine cells throughout the body. These tumors have an increasing incidence of 2.5–5/100,000 people per year ( Kloppel & Anlauf 2005

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-18-0494
Volume/Issue:
Volume 26: Issue 3
Free access
S Felder Medizinische Klinik mit Schwerpunkt Hepatologie und Gastroenterologie (einschl. Arbeitsbereich Stoffwechselerkrankungen), Charité – Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany

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H Jann Medizinische Klinik mit Schwerpunkt Hepatologie und Gastroenterologie (einschl. Arbeitsbereich Stoffwechselerkrankungen), Charité – Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany

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R Arsenic Institut für Pathologie, Charité – Universitätsmedizin Berlin, Campus Mitte, Berlin, Germany

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T Denecke Klinik für Radiologie, Charité – Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany

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V Prasad Klinik für Nuklearmedizin, Charité – Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany
Klinik für Nuklearmedizin, Universitätklinikum Ulm, Ulm, Germany

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B Knappe-Drzikova Medizinische Klinik mit Schwerpunkt Hepatologie und Gastroenterologie (einschl. Arbeitsbereich Stoffwechselerkrankungen), Charité – Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany

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S Maasberg Medizinische Klinik mit Schwerpunkt Hepatologie und Gastroenterologie (einschl. Arbeitsbereich Stoffwechselerkrankungen), Charité – Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany
Innere Medizin und Gastroenterologie, Asklepios Klinik St. Georg, Asklepios Medical School, Hamburg, Germany

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B Wiedenmann Medizinische Klinik mit Schwerpunkt Hepatologie und Gastroenterologie (einschl. Arbeitsbereich Stoffwechselerkrankungen), Charité – Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany

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M Pavel Medizinische Klinik mit Schwerpunkt Hepatologie und Gastroenterologie (einschl. Arbeitsbereich Stoffwechselerkrankungen), Charité – Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany
Medizinische Klinik 1, Gastroenterologie, Pneumologie und Endokrinologie, Universitätsklinikum der Friedrich-Alexander Universität Erlangen, Erlangen, Germany

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A Pascher Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Charité – Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany
Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Uinversitätsklinikum Münster, Münster, Germany

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U F Pape Medizinische Klinik mit Schwerpunkt Hepatologie und Gastroenterologie (einschl. Arbeitsbereich Stoffwechselerkrankungen), Charité – Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin, Germany
Innere Medizin und Gastroenterologie, Asklepios Klinik St. Georg, Asklepios Medical School, Hamburg, Germany

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Introduction Gastric neuroendocrine neoplasias (gNENs) are a heterogeneous subgroup of gastroenteropancreatic neuroendocrine neoplasias (GEP-NENs) derived from specialized cells of the diffuse endocrine system and were initially described as

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-18-0582
Volume/Issue:
Volume 26: Issue 9
Open access
Eamonn R Maher Department of Medical Genetics, Department of Clinical Genetics, University of Cambridge, Cambridge, UK and
Department of Medical Genetics, Department of Clinical Genetics, University of Cambridge, Cambridge, UK and

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studies of HIF2A mutations in pheochromocytoma and paraganglioma have provided novel insights into the role of hypoxic gene response pathways in the pathogenesis of endocrine neoplasia. To date, a number of common themes are emerging. First, most HIF2A

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-13-0146
Volume/Issue:
Volume 20: Issue 3
Free access