{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T18:23:10Z","timestamp":1792174990939,"version":"4.4.0"},"reference-count":63,"publisher":"The Endocrine Society","issue":"10","license":[{"start":{"date-parts":[[2023,3,22]],"date-time":"2023-03-22T00:00:00Z","timestamp":1679443200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"German Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"name":"University Medicine Zurich"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,9,18]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Context<\/jats:title>\n                    <jats:p>Pheochromocytomas and paragangliomas (PPGLs) with pathogenic mutations in the succinate dehydrogenase subunit B (SDHB) are associated with a high metastatic risk. Somatostatin receptor 2 (SSTR2)\u2013dependent imaging is the most sensitive imaging modality for SDHB-related PPGLs, suggesting that SSTR2 expression is a significant cell surface therapeutic biomarker of such tumors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Exploration of the relationship between SSTR2 immunoreactivity and SDHB immunoreactivity, mutational status, and clinical behavior of PPGLs. Evaluation of SSTR-based therapies in metastatic PPGLs.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Retrospective analysis of a multicenter cohort of PPGLs at 6 specialized Endocrine Tumor Centers in Germany, The Netherlands, and Switzerland. Patients with PPGLs participating in the ENSAT registry were included. Clinical data were extracted from medical records, and immunohistochemistry (IHC) for SDHB and SSTR2 was performed in patients with available tumor tissue. Immunoreactivity of SSTR2 was investigated using Volante scores. The main outcome measure was the association of SSTR2 IHC positivity with genetic and clinical\u2013pathological features of PPGLs.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of 202 patients with PPGLs, 50% were SSTR2 positive. SSTR2 positivity was significantly associated with SDHB- and SDHx-related PPGLs, with the strongest SSTR2 staining intensity in SDHB-related PPGLs (P = .01). Moreover, SSTR2 expression was significantly associated with metastatic disease independent of SDHB\/SDHx mutation status (P &amp;lt; .001). In metastatic PPGLs, the disease control rate with first-line SSTR-based radionuclide therapy was 67% (n = 22, n = 11 SDHx), and with first-line \u201ccold\u201d somatostatin analogs 100% (n = 6, n = 3 SDHx).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>SSTR2 expression was independently associated with SDHB\/SDHx mutations and metastatic disease. We confirm a high disease control rate of somatostatin receptor\u2013based therapies in metastatic PPGLs.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1210\/clinem\/dgad166","type":"journal-article","created":{"date-parts":[[2023,3,22]],"date-time":"2023-03-22T06:50:57Z","timestamp":1679467857000},"page":"2676-2685","source":"Crossref","is-referenced-by-count":51,"title":["Metastatic Pheochromocytoma and Paraganglioma: Somatostatin Receptor 2 Expression, Genetics, and Therapeutic Responses"],"prefix":"10.1210","volume":"108","author":[{"given":"Alessa","family":"Fischer","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), and University of Zurich (UZH) , CH-8091 Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Kloos","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), and University of Zurich (UZH) , CH-8091 Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Umberto","family":"Maccio","sequence":"additional","affiliation":[{"name":"Department of Pathology and Molecular Pathology, University Hospital Zurich , CH-8091 Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliane","family":"Friemel","sequence":"additional","affiliation":[{"name":"Department of Pathology and Molecular Pathology, University Hospital Zurich , CH-8091 Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanna","family":"Remde","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of W\u00fcrzburg , 97080 W\u00fcrzburg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Fassnacht","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of W\u00fcrzburg , 97080 W\u00fcrzburg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Pamporaki","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine III, University Hospital Carl Gustav Carus, Technische Universit\u00e4t Dresden , 01307 Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graeme","family":"Eisenhofer","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine III, University Hospital Carl Gustav Carus, Technische Universit\u00e4t Dresden , 01307 Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henri J L M","family":"Timmers","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Department of Internal Medicine, Radboud University Medical Center , 6525 GA Nijmegen , Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mercedes","family":"Robledo","sequence":"additional","affiliation":[{"name":"Hereditary Endocrine Cancer Group, Spanish National Cancer Research Center (CNIO) , Madrid , Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras , Madrid , Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie M J","family":"Fliedner","sequence":"additional","affiliation":[{"name":"First Department of Medicine, University Medical Center Schleswig-Holstein , 23538 L\u00fcbeck , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katharina","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, University Hospital, Ludwig-Maximilians-University Munich , 80336 Munich , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian","family":"Maurer","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, University Hospital, Ludwig-Maximilians-University Munich , 80336 Munich , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Astrid","family":"Reul","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), and University of Zurich (UZH) , CH-8091 Zurich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathrin","family":"Zitzmann","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, University Hospital, Ludwig-Maximilians-University Munich , 80336 Munich , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Bechmann","sequence":"additional","affiliation":[{"name":"Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universit\u00e4t Dresden, Fetscherstrasse , 01307 Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gintar\u0117","family":"\u017dygien\u0117","sequence":"additional","affiliation":[{"name":"Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universit\u00e4t Dresden, Fetscherstrasse , 01307 Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Richter","sequence":"additional","affiliation":[{"name":"Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universit\u00e4t Dresden, Fetscherstrasse , 01307 Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Constanze","family":"Hantel","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), and University of Zurich (UZH) , CH-8091 Zurich , Switzerland"},{"name":"Department of Internal Medicine III, University Hospital Carl Gustav Carus, Technische Universit\u00e4t Dresden , 01307 Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Vetter","sequence":"additional","affiliation":[{"name":"Department of Visceral and Transplantation Surgery, University Hospital , 8091 Z\u00fcrich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kuno","family":"Lehmann","sequence":"additional","affiliation":[{"name":"Department of Visceral and Transplantation Surgery, University Hospital , 8091 Z\u00fcrich , Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hermine","family":"Mohr","sequence":"additional","affiliation":[{"name":"Institute for Diabetes and Cancer, Helmholtz Zentrum M\u00fcnchen , 85764 Neuherberg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia S","family":"Pellegata","sequence":"additional","affiliation":[{"name":"Institute for Diabetes and Cancer, Helmholtz Zentrum M\u00fcnchen , 85764 Neuherberg , Germany"},{"name":"Department of Biology and Biotechnology, University of Pavia , 27100 Pavia , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Ullrich","sequence":"additional","affiliation":[{"name":"Department of Radiopharmaceutical and Chemical Biology, Institute of Radiopharmaceutical Cancer Research, Helmholtz-Zentrum Dresden-Rossendorf (HZDR) , Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens","family":"Pietzsch","sequence":"additional","affiliation":[{"name":"Department of Radiopharmaceutical and Chemical Biology, Institute of Radiopharmaceutical Cancer Research, Helmholtz-Zentrum Dresden-Rossendorf (HZDR) , Dresden , Germany"},{"name":"Faculty of Chemistry and Food Chemistry, School of Science, Technische Universit\u00e4t Dresden , Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian G","family":"Ziegler","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of W\u00fcrzburg , 97080 W\u00fcrzburg , Germany"},{"name":"Department of Internal Medicine III, University Hospital Carl Gustav Carus, Technische Universit\u00e4t Dresden , 01307 Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan R","family":"Bornstein","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), and University of Zurich (UZH) , CH-8091 Zurich , Switzerland"},{"name":"Department of Internal Medicine III, University Hospital Carl Gustav Carus, Technische Universit\u00e4t Dresden , 01307 Dresden , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Kroiss","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, University Hospital, Ludwig-Maximilians-University Munich , 80336 Munich , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Reincke","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, University Hospital, Ludwig-Maximilians-University Munich , 80336 Munich , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karel","family":"Pacak","sequence":"additional","affiliation":[{"name":"Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health , Rockville, MD 20847 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley B","family":"Grossman","sequence":"additional","affiliation":[{"name":"Green Templeton College, University of Oxford , Oxford , UK"},{"name":"NET Unit, ENETS Centre of Excellence, Royal Free Hospital , London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix","family":"Beuschlein","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), and University of Zurich (UZH) , CH-8091 Zurich , Switzerland"},{"name":"Department of Medicine IV, University Hospital, Ludwig-Maximilians-University Munich , 80336 Munich , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7064-590X","authenticated-orcid":false,"given":"Svenja","family":"N\u00f6lting","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), and University of Zurich (UZH) , CH-8091 Zurich , Switzerland"},{"name":"Department of Medicine IV, University Hospital, Ludwig-Maximilians-University Munich , 80336 Munich , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2023,3,22]]},"reference":[{"issue":"2","key":"2023091800453884100_dgad166-B1","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1038\/nrc3648","article-title":"Pheochromocytoma and paraganglioma pathogenesis: learning from genetic heterogeneity","volume":"14","author":"Dahia","year":"2014","journal-title":"Nat Rev Cancer"},{"issue":"5","key":"2023091800453884100_dgad166-B2","doi-asserted-by":"crossref","first-page":"1338","DOI":"10.1245\/s10434-020-08221-2","article-title":"Update on pheochromocytoma and paraganglioma from the SSO endocrine and head and neck disease site working group, part 2 of 2: perioperative management and outcomes of pheochromocytoma and paraganglioma","volume":"27","author":"Patel","year":"2020","journal-title":"Ann Surg Oncol"},{"issue":"2","key":"2023091800453884100_dgad166-B3","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1210\/endrev\/bnab019","article-title":"Personalized management of pheochromocytoma and paraganglioma","volume":"43","author":"N\u00f6lting","year":"2021","journal-title":"Endocr Rev"},{"issue":"6","key":"2023091800453884100_dgad166-B4","doi-asserted-by":"crossref","first-page":"2367","DOI":"10.1210\/jc.2018-01968","article-title":"Prognosis of malignant pheochromocytoma and paraganglioma (MAPP-prono study): a European network for the study of adrenal tumors retrospective study","volume":"104","author":"Hescot","year":"2019","journal-title":"J Clin Endocrinol Metab"},{"issue":"8","key":"2023091800453884100_dgad166-B5","doi-asserted-by":"crossref","first-page":"1443","DOI":"10.1097\/HJH.0000000000002438","article-title":"Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma: a position statement and consensus of the working group on endocrine hypertension of the European Society of hypertension","volume":"38","author":"Lenders","year":"2020","journal-title":"J Hypertens"},{"issue":"2","key":"2023091800453884100_dgad166-B6","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/j.ccell.2017.01.001","article-title":"Comprehensive molecular characterization of pheochromocytoma and paraganglioma","volume":"31","author":"Fishbein","year":"2017","journal-title":"Cancer Cell"},{"issue":"1","key":"2023091800453884100_dgad166-B7","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1155\/2015\/138573","article-title":"Profiling of somatic mutations in phaeochromocytoma and paraganglioma by targeted next generation sequencing analysis","volume":"2015","author":"Luchetti","year":"2015","journal-title":"Int J Endocrinol"},{"issue":"10","key":"2023091800453884100_dgad166-B8","doi-asserted-by":"crossref","first-page":"3295","DOI":"10.1210\/clinem\/dgaa502","article-title":"Sino-European differences in the genetic landscape and clinical presentation of pheochromocytoma and paraganglioma","volume":"105","author":"Jiang","year":"2020","journal-title":"J Clin Endocrinol Metab"},{"issue":"3","key":"2023091800453884100_dgad166-B9","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1097\/MED.0000000000000634","article-title":"Genetics of pheochromocytoma and paraganglioma","volume":"28","author":"Wachtel","year":"2021","journal-title":"Curr Opin Endocrinol Diabetes Obes"},{"issue":"5","key":"2023091800453884100_dgad166-B10","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1530\/ERC-19-0024","article-title":"Genotype\u2013phenotype correlations in pheochromocytoma and paraganglioma: a systematic review and individual patient meta-analysis","volume":"26","author":"Crona","year":"2019","journal-title":"Endocr Relat Cancer"},{"issue":"11","key":"2023091800453884100_dgad166-B11","doi-asserted-by":"crossref","first-page":"R627","DOI":"10.1530\/ERC-19-0165","article-title":"Molecular imaging and radionuclide therapy of pheochromocytoma and paraganglioma in the era of genomic characterization of disease subgroups","volume":"26","author":"Ta\u00efeb","year":"2019","journal-title":"Endocr Relat Cancer"},{"issue":"8","key":"2023091800453884100_dgad166-B12","doi-asserted-by":"crossref","first-page":"764","DOI":"10.1016\/S1470-2045(09)70164-0","article-title":"An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis","volume":"10","author":"van Nederveen","year":"2009","journal-title":"Lancet Oncol"},{"issue":"6","key":"2023091800453884100_dgad166-B13","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1038\/modpathol.2015.41","article-title":"SDHB\/SDHA immunohistochemistry in pheochromocytomas and paragangliomas: a multicenter interobserver variation analysis using virtual microscopy: a multinational study of the European network for the study of adrenal tumors (ENS@T)","volume":"28","author":"Papathomas","year":"2015","journal-title":"Mod Pathol"},{"issue":"3","key":"2023091800453884100_dgad166-B14","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1006\/frne.1999.0183","article-title":"Somatostatin and its receptor family","volume":"20","author":"Patel","year":"1999","journal-title":"Front Neuroendocrinol"},{"issue":"6","key":"2023091800453884100_dgad166-B15","doi-asserted-by":"crossref","first-page":"850","DOI":"10.1111\/j.1365-2265.2007.03124.x","article-title":"Immunohistochemical localization of somatostatin receptor subtypes in benign and malignant adrenal tumours","volume":"68","author":"Unger","year":"2008","journal-title":"Clin Endocrinol (Oxf)"},{"issue":"37","key":"2023091800453884100_dgad166-B16","doi-asserted-by":"crossref","first-page":"15879","DOI":"10.1073\/pnas.0907843106","article-title":"Expression of neuropeptide hormone receptors in human adrenal tumors and cell lines: antiproliferative effects of peptide analogues","volume":"106","author":"Ziegler","year":"2009","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"7","key":"2023091800453884100_dgad166-B17","doi-asserted-by":"crossref","first-page":"836","DOI":"10.1007\/s002590100541","article-title":"Somatostatin receptor sst1\u2013sst5 expression in normal and neoplastic human tissues using receptor autoradiography with subtype-selective ligands","volume":"28","author":"Reubi","year":"2001","journal-title":"Eur J Nucl Med"},{"issue":"10","key":"2023091800453884100_dgad166-B18","doi-asserted-by":"crossref","first-page":"2112","DOI":"10.1007\/s00259-019-04398-1","article-title":"European Association of nuclear medicine practice guideline\/society of nuclear medicine and molecular imaging procedure standard 2019 for radionuclide imaging of phaeochromocytoma and paraganglioma","volume":"46","author":"Ta\u00efeb","year":"2019","journal-title":"Eur J Nucl Med Mol Imaging"},{"issue":"6","key":"2023091800453884100_dgad166-B19","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1080\/02841860701441848","article-title":"Peptide receptor radionuclide therapy with radiolabelled somatostatin analogues in patients with somatostatin receptor positive tumours","volume":"46","author":"Van Essen","year":"2007","journal-title":"Acta Oncol"},{"issue":"17","key":"2023091800453884100_dgad166-B20","doi-asserted-by":"crossref","first-page":"3888","DOI":"10.1158\/1078-0432.CCR-14-2751","article-title":"Superiority of [68Ga]-DOTATATE PET\/CT to other functional imaging modalities in the localization of SDHB -associated metastatic pheochromocytoma and paraganglioma","volume":"21","author":"Janssen","year":"2015","journal-title":"Clin Cancer Res"},{"issue":"3","key":"2023091800453884100_dgad166-B21","doi-asserted-by":"crossref","first-page":"369","DOI":"10.2967\/jnumed.118.211706","article-title":"Performance of 68 Ga-DOTA\u2013conjugated somatostatin receptor\u2013targeting peptide PET in detection of pheochromocytoma and paraganglioma: a systematic review and metaanalysis","volume":"60","author":"Han","year":"2019","journal-title":"J Nucl Med"},{"issue":"1","key":"2023091800453884100_dgad166-B22","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1186\/s40644-016-0084-2","article-title":"68Ga-DOTATATE And 18F-FDG PET\/CT in paraganglioma and pheochromocytoma: utility, patterns and heterogeneity","volume":"16","author":"Chang","year":"2016","journal-title":"Cancer Imaging"},{"issue":"4","key":"2023091800453884100_dgad166-B23","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1007\/s11307-010-0396-8","article-title":"68Ga-DOTA-TATE PET vs. 123I-MIBG in identifying malignant neural crest tumours","volume":"13","author":"Naji","year":"2011","journal-title":"Mol Imaging Biol"},{"key":"2023091800453884100_dgad166-B24","doi-asserted-by":"crossref","first-page":"625312","DOI":"10.3389\/fendo.2021.625312","article-title":"Somatostatin receptors and analogs in pheochromocytoma and paraganglioma: old players in a new precision medicine world","volume":"12","author":"Patel","year":"2021","journal-title":"Front Endocrinol"},{"issue":"11","key":"2023091800453884100_dgad166-B25","doi-asserted-by":"crossref","first-page":"2963","DOI":"10.1210\/clinem\/dgac471","article-title":"Targeted therapies in pheochromocytoma and paraganglioma","volume":"107","author":"Wang","year":"2022","journal-title":"J Clin Endocrinol Metab"},{"issue":"2","key":"2023091800453884100_dgad166-B26","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1056\/NEJMoa1607427","article-title":"Phase 3 trial of 177 Lu-Dotatate for midgut neuroendocrine tumors","volume":"376","author":"Strosberg","year":"2017","journal-title":"N Engl J Med"},{"key":"2023091800453884100_dgad166-B27","author":"European network for the study of adrenal tumors (ENS@T)"},{"issue":"1","key":"2023091800453884100_dgad166-B28","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1530\/EJE-20-1407","article-title":"Mass spectrometry imaging identifies metabolic patterns associated with malignant potential in pheochromocytoma and paraganglioma","volume":"185","author":"Murakami","year":"2021","journal-title":"Eur J Endocrinol"},{"issue":"11","key":"2023091800453884100_dgad166-B29","doi-asserted-by":"crossref","first-page":"1172","DOI":"10.1038\/modpathol.3800954","article-title":"Somatostatin receptor type 2A immunohistochemistry in neuroendocrine tumors: a proposal of scoring system correlated with somatostatin receptor scintigraphy","volume":"20","author":"Volante","year":"2007","journal-title":"Mod Pathol"},{"key":"2023091800453884100_dgad166-B30","author":"Fischer"},{"issue":"6","key":"2023091800453884100_dgad166-B31","doi-asserted-by":"crossref","first-page":"809","DOI":"10.3390\/cancers11060809","article-title":"Optimizing genetic workup in pheochromocytoma and paraganglioma by integrating diagnostic and research approaches","volume":"11","author":"Gieldon","year":"2019","journal-title":"Cancers"},{"issue":"6","key":"2023091800453884100_dgad166-B32","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1530\/ERC-21-0355","article-title":"Personalized drug testing in human pheochromocytoma\/paraganglioma primary cultures","volume":"29","author":"Wang","year":"2022","journal-title":"Endocr Relat Cancer."},{"issue":"5","key":"2023091800453884100_dgad166-B33","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1038\/gim.2015.30","article-title":"Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American college of medical genetics and genomics and the association for molecular pathology","volume":"17","author":"Richards","year":"2015","journal-title":"Genet Med"},{"issue":"1","key":"2023091800453884100_dgad166-B34","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1186\/1471-2407-14-523","article-title":"The size of the primary tumor and age at initial diagnosis are independent predictors of the metastatic behavior and survival of patients with SDHB-related pheochromocytoma and paraganglioma: a retrospective cohort study","volume":"14","author":"Schovanek","year":"2014","journal-title":"BMC Cancer"},{"issue":"11","key":"2023091800453884100_dgad166-B35","doi-asserted-by":"crossref","first-page":"1739","DOI":"10.1016\/j.ejca.2011.07.016","article-title":"Plasma methoxytyramine: a novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status","volume":"48","author":"Eisenhofer","year":"2012","journal-title":"Eur J Cancer"},{"issue":"July 2022","key":"2023091800453884100_dgad166-B36","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1016\/j.ejca.2022.03.032","article-title":"Determinants of disease-specific survival in patients with and without metastatic pheochromocytoma and paraganglioma","volume":"169","author":"Pamporaki","year":"2022","journal-title":"Eur J Cancer"},{"key":"2023091800453884100_dgad166-B37","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.humpath.2018.11.020","article-title":"Variable somatostatin receptor subtype expression in 151 primary pheochromocytomas and paragangliomas","volume":"86","author":"Leijon","year":"2019","journal-title":"Hum Pathol"},{"issue":"3","key":"2023091800453884100_dgad166-B38","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1016\/j.humpath.2014.11.012","article-title":"Increased SSTR2A and SSTR3 expression in succinate dehydrogenase\u2013deficient pheochromocytomas and paragangliomas","volume":"46","author":"Elston","year":"2015","journal-title":"Hum Pathol"},{"issue":"2","key":"2023091800453884100_dgad166-B39","doi-asserted-by":"crossref","first-page":"135","DOI":"10.3233\/JCB-199005","article-title":"Fluorescent mouse pheochromocytoma spheroids expressing hypoxia-inducible factor 2 alpha: morphologic and radiopharmacologic characterization","volume":"5","author":"Seifert","year":"2019","journal-title":"J Cell Biotechnol"},{"issue":"12","key":"2023091800453884100_dgad166-B40","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1530\/ERC-16-0231","article-title":"Novel insights into the polycythemia\u2013paraganglioma\u2013somatostatinoma syndrome","volume":"23","author":"D\u00e4rr","year":"2016","journal-title":"Endocr Relat Cancer"},{"issue":"1","key":"2023091800453884100_dgad166-B41","doi-asserted-by":"crossref","first-page":"278","DOI":"10.7150\/thno.77918","article-title":"Epigenetic drugs in somatostatin type 2 receptor radionuclide theranostics and radiation transcriptomics in mouse pheochromocytoma models","volume":"13","author":"Ullrich","year":"2023","journal-title":"Theranostics"},{"issue":"10","key":"2023091800453884100_dgad166-B42","doi-asserted-by":"crossref","first-page":"1505","DOI":"10.3390\/cancers11101505","article-title":"Current management of pheochromocytoma\/paraganglioma: a guide for the practicing clinician in the era of precision medicine","volume":"11","author":"N\u00f6lting","year":"2019","journal-title":"Cancers"},{"issue":"4","key":"2023091800453884100_dgad166-B43","first-page":"334","article-title":"Radiolabeled DOTATOC in patients with advanced paraganglioma and pheochromocytoma","volume":"52","author":"Forrer","year":"2008","journal-title":"Q J Nucl Med Mol Imaging"},{"issue":"6","key":"2023091800453884100_dgad166-B44","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1111\/cen.14106","article-title":"Peptide receptor radionuclide therapy in the management of advanced pheochromocytoma and paraganglioma: a systematic review and meta-analysis","volume":"91","author":"Satapathy","year":"2019","journal-title":"Clin Endocrinol (Oxf)"},{"issue":"7","key":"2023091800453884100_dgad166-B45","doi-asserted-by":"crossref","first-page":"909","DOI":"10.3390\/cancers11070909","article-title":"Favorable outcome in patients with pheochromocytoma and paraganglioma treated with 177Lu-DOTATATE","volume":"11","author":"Vyakaranam","year":"2019","journal-title":"Cancers"},{"issue":"9","key":"2023091800453884100_dgad166-B46","doi-asserted-by":"crossref","first-page":"3278","DOI":"10.1210\/jc.2017-00816","article-title":"Efficacy of peptide receptor radionuclide therapy for functional metastatic paraganglioma and pheochromocytoma","volume":"102","author":"Kong","year":"2017","journal-title":"J Clin Endocrinol Metab"},{"issue":"9","key":"2023091800453884100_dgad166-B47","doi-asserted-by":"crossref","first-page":"864","DOI":"10.1530\/EC-20-0292","article-title":"177Lu-DOTATATE therapy in metastatic\/inoperable pheochromocytoma-paraganglioma","volume":"9","author":"Jaiswal","year":"2020","journal-title":"Endocr Connect"},{"issue":"4","key":"2023091800453884100_dgad166-B48","doi-asserted-by":"crossref","first-page":"100171","DOI":"10.1016\/j.esmoop.2021.100171","article-title":"Peptide receptor radionuclide therapy in patients with metastatic progressive pheochromocytoma and paraganglioma: long-term toxicity, efficacy and prognostic biomarker data of phase II clinical trials","volume":"6","author":"Severi","year":"2021","journal-title":"ESMO Open"},{"issue":"4","key":"2023091800453884100_dgad166-B49","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1002\/jso.24553","article-title":"Peptide receptor radionuclide treatment and (131)I-MIBG in the management of patients with metastatic\/progressive phaeochromocytomas and paragangliomas","volume":"115","author":"Nastos","year":"2017","journal-title":"J Surg Oncol"},{"issue":"05","key":"2023091800453884100_dgad166-B50","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1055\/s-0032-1311637","article-title":"Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE in individuals with neck or mediastinal paraganglioma (PGL)","volume":"44","author":"Zovato","year":"2012","journal-title":"Horm Metab Res"},{"issue":"5","key":"2023091800453884100_dgad166-B51","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1007\/s12032-016-0737-9","article-title":"Peptide receptor radionuclide therapy for metastatic paragangliomas","volume":"33","author":"Pinato","year":"2016","journal-title":"Med Oncol"},{"issue":"8","key":"2023091800453884100_dgad166-B52","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.1007\/s00259-015-3029-2","article-title":"Peptide receptor radionuclide therapy with 90Y\/177Lu-labelled peptides for inoperable head and neck paragangliomas (glomus tumours)","volume":"42","author":"Puranik","year":"2015","journal-title":"Eur J Nucl Med Mol Imaging"},{"issue":"1","key":"2023091800453884100_dgad166-B53","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1186\/s13550-019-0484-y","article-title":"Concomitant 177Lu-DOTATATE and capecitabine therapy in malignant paragangliomas","volume":"9","author":"Yadav","year":"2019","journal-title":"EJNMMI Res"},{"issue":"17","key":"2023091800453884100_dgad166-B54","doi-asserted-by":"crossref","first-page":"2416","DOI":"10.1200\/JCO.2010.33.7873","article-title":"Response, survival, and long-term toxicity after therapy with the radiolabeled somatostatin analogue [90 Y-DOTA]-TOC in metastasized neuroendocrine cancers","volume":"29","author":"Imhof","year":"2011","journal-title":"J Clin Oncol"},{"issue":"1","key":"2023091800453884100_dgad166-B55","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1530\/EJE-18-0901","article-title":"Treatment of inoperable or metastatic paragangliomas and pheochromocytomas with peptide receptor radionuclide therapy using 177Lu-DOTATATE","volume":"181","author":"Zandee","year":"2019","journal-title":"Eur J Endocrinol"},{"issue":"5","key":"2023091800453884100_dgad166-B56","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1507\/endocrj.50.507","article-title":"Case report: patient with multiple paragangliomas treated with long acting somatostatin analogue","volume":"50","author":"Tonyukuk","year":"2003","journal-title":"Endocr J"},{"issue":"12","key":"2023091800453884100_dgad166-B57","doi-asserted-by":"crossref","first-page":"E391","DOI":"10.1002\/hed.23348","article-title":"Effects of octreotide therapy in progressive head and neck paragangliomas: case series: octreotide in head and neck paragangliomas","volume":"35","author":"van Hulsteijn","year":"2013","journal-title":"Head Neck"},{"issue":"1","key":"2023091800453884100_dgad166-B58","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1007\/s13139-019-00629-3","article-title":"Role of 68Ga-DOTATATE PET\/CT in a case of SDHB-related pterygopalatine fossa paraganglioma successfully controlled with octreotide","volume":"54","author":"Jha","year":"2020","journal-title":"Nucl Med Mol Imaging"},{"issue":"9","key":"2023091800453884100_dgad166-B59","doi-asserted-by":"crossref","first-page":"1768","DOI":"10.1136\/gutjnl-2020-321300","article-title":"Treatment of advanced gastroenteropancreatic neuroendocrine neoplasia, are we on the way to personalised medicine?","volume":"70","author":"Rinke","year":"2021","journal-title":"Gut"},{"issue":"3","key":"2023091800453884100_dgad166-B60","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1056\/NEJMoa1316158","article-title":"Lanreotide in metastatic enteropancreatic neuroendocrine tumors","volume":"371","author":"Caplin","year":"2014","journal-title":"N Engl J Med"},{"issue":"28","key":"2023091800453884100_dgad166-B61","doi-asserted-by":"crossref","first-page":"4656","DOI":"10.1200\/JCO.2009.22.8510","article-title":"Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID study group","volume":"27","author":"Rinke","year":"2009","journal-title":"J Clin Oncol"},{"issue":"3","key":"2023091800453884100_dgad166-B62","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1159\/000289569","article-title":"VEGF Secretion by neuroendocrine tumor cells is inhibited by octreotide and by inhibitors of the PI3K\/AKT\/mTOR pathway","volume":"91","author":"Villaume","year":"2010","journal-title":"Neuroendocrinology"},{"issue":"12","key":"2023091800453884100_dgad166-B63","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1530\/ERC-18-0136","article-title":"Strain-specific metastatic phenotypes in pheochromocytoma allograft mice","volume":"25","author":"Ullrich","year":"2018","journal-title":"Endocr Relat Cancer"}],"container-title":["The Journal of Clinical Endocrinology &amp; Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jcem\/advance-article-pdf\/doi\/10.1210\/clinem\/dgad166\/50522564\/dgad166.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jcem\/article-pdf\/108\/10\/2676\/51533111\/dgad166.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jcem\/article-pdf\/108\/10\/2676\/51533111\/dgad166.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,17]],"date-time":"2023-09-17T20:46:33Z","timestamp":1694983593000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jcem\/article\/108\/10\/2676\/7083389"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,22]]},"references-count":63,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2023,3,22]]},"published-print":{"date-parts":[[2023,9,18]]}},"URL":"https:\/\/doi.org\/10.1210\/clinem\/dgad166","relation":{},"ISSN":["0021-972X","1945-7197"],"issn-type":[{"value":"0021-972X","type":"print"},{"value":"1945-7197","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,10]]},"published":{"date-parts":[[2023,3,22]]}}}