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Yuanliang Li Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing University of Chinese Medicine, Beijing, China

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Yiying Guo Department of Integrative Oncology, China-Japan Friendship Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Zixuan Cheng Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing University of Chinese Medicine, Beijing, China

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Chao Tian Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing University of Chinese Medicine, Beijing, China

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Yingying Chen Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing University of Chinese Medicine, Beijing, China

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Ruao Chen Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing University of Chinese Medicine, Beijing, China

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Fuhuan Yu Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing University of Chinese Medicine, Beijing, China

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Yanfen Shi Department of Pathology, China-Japan Friendship Hospital, Beijing, China

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Fei Su Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing, China

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Shuhua Zhao Department of Biological Information Research, HaploX Biotechnology Co., Ltd, Shenzhen, Guangdong, China

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Zhizheng Wang Academic Department, HaploX Biotechnology, Co., Ltd, Shenzhen, Guangdong, China

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Jie Luo Department of Pathology, China-Japan Friendship Hospital, Beijing, China

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Huangying Tan Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing University of Chinese Medicine, Beijing, China
Department of Integrative Oncology, China-Japan Friendship Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing, China

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). According to the differentiation, they can be classified as well-differentiated rectal neuroendocrine tumors (R-NETs) and poorly differentiated neuroendocrine carcinomas (R-NECs). In recent years, several drugs or treatment strategies have been developed for

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Nicole Panarelli Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York, USA

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Kathrin Tyryshkin Laboratory of Translational RNA Biology, Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada

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Justin Jong Mun Wong Laboratory of Translational RNA Biology, Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada

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Adrianna Majewski Laboratory of Translational RNA Biology, Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada

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Xiaojing Yang Laboratory of Translational RNA Biology, Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada

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Theresa Scognamiglio Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York, USA

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Michelle Kang Kim Center for Carcinoid and Neuroendocrine Tumors of Mount Sinai, Icahn School of Medicine at Mount Sinai, New York, New York, USA

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Kimberly Bogardus HHMI, Laboratory of RNA Molecular Biology, The Rockefeller University, New York, New York, USA

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Thomas Tuschl HHMI, Laboratory of RNA Molecular Biology, The Rockefeller University, New York, New York, USA

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Yao-Tseng Chen Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York, USA

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Neil Renwick Laboratory of Translational RNA Biology, Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada
HHMI, Laboratory of RNA Molecular Biology, The Rockefeller University, New York, New York, USA

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Digestive Tract ( Bosman et al . 2010 ). Four additional cases of low-grade rectal carcinoid tumors were obtained (through MK) from the Center for Carcinoid and Neuroendocrine Tumors of Mount Sinai, Icahn School of Medicine at Mount Sinai. Representative

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Matthew H Kulke Department of Medical Oncology, Dana-Farber Cancer Institute and Harvard Medical School, Dana-Farber Cancer Institute, 44 Binney Street, Boston, Massachusetts 02115, USA

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Introduction Neuroendocrine tumors comprise a diverse group of malignancies and, when defined broadly, include not only gastrointestinal neuroendocrine tumors but also pheochromocytoma, thyroid cancer, and even small cell lung

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Guido Rindi Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
Università Cattolica del Sacro Cuore, Rome, Italy
ENETS Center of Excellence, Neuroendocrine Tumour (NET) Center, Rome, Italy

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Frediano Inzani Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
ENETS Center of Excellence, Neuroendocrine Tumour (NET) Center, Rome, Italy

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based on the common expression of the general markers of neuroendocrine differentiation chromogranin A and synaptophysin. NEN definition embraces the two families (classes) of the well-differentiated lesions, defined as neuroendocrine tumor (NET), and

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Yeting Du Harvard School of Public Health, Boston, Massachusetts, USA

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Monica Ter-Minassian Harvard School of Public Health, Boston, Massachusetts, USA
Dana-Farber Cancer Institute, Boston, Massachusetts

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Lauren Brais Dana-Farber Cancer Institute, Boston, Massachusetts

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Nichole Brooks Dana-Farber Cancer Institute, Boston, Massachusetts

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Amanda Waldron Dana-Farber Cancer Institute, Boston, Massachusetts

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Jennifer A Chan Dana-Farber Cancer Institute, Boston, Massachusetts

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Xihong Lin Harvard School of Public Health, Boston, Massachusetts, USA

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Peter Kraft Harvard School of Public Health, Boston, Massachusetts, USA

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David C Christiani Harvard School of Public Health, Boston, Massachusetts, USA
Massachusetts General Hospital, Boston, Massachusetts, USA

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Matthew H Kulke Dana-Farber Cancer Institute, Boston, Massachusetts

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lungs and bronchi, which account for up to 30% of all neuroendocrine tumors, with small intestine, appendix and rectal carcinoid tumors comprising the majority of the remaining sites ( Yao et al . 2008 ). Within the gastrointestinal tract, carcinoid

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M Fraenkel
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M Kim Endocrinology, Mount Sinai Medical Center, Department of Clinical Medicine and Surgery, Endocrinology, Erasmus MC, Department of Internal Medicine, Soroka University Medical Center and the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheeba, Israel

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A Faggiano Endocrinology, Mount Sinai Medical Center, Department of Clinical Medicine and Surgery, Endocrinology, Erasmus MC, Department of Internal Medicine, Soroka University Medical Center and the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheeba, Israel
Endocrinology, Mount Sinai Medical Center, Department of Clinical Medicine and Surgery, Endocrinology, Erasmus MC, Department of Internal Medicine, Soroka University Medical Center and the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheeba, Israel

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W W de Herder Endocrinology, Mount Sinai Medical Center, Department of Clinical Medicine and Surgery, Endocrinology, Erasmus MC, Department of Internal Medicine, Soroka University Medical Center and the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheeba, Israel

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G D Valk Endocrinology, Mount Sinai Medical Center, Department of Clinical Medicine and Surgery, Endocrinology, Erasmus MC, Department of Internal Medicine, Soroka University Medical Center and the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheeba, Israel

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On behalf of the Knowledge NETwork
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’(Mesh)) OR ‘Prevalence’(Mesh)) AND ((‘Neuroendocrine Tumors’(Mesh) OR ‘Carcinoma, Neuroendocrine’(Mesh) OR ‘Gastro-enteropancreatic neuroendocrine tumor’ (Supplementary Concept)) OR ((neuroendocrine AND (tumors OR tumour)) OR (neuro-endocrine AND (tumors OR

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Aura D Herrera-Martínez Division of Endocrinology, Department of Internal Medicine, Erasmus MC, University Medical Center Rotterdam, Rotterdam, the Netherlands
Maimonides Institute for Biomedical Research of Cordoba (IMIBIC); Reina Sofia University Hospital, Córdoba, Spain

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Leo J Hofland Division of Endocrinology, Department of Internal Medicine, Erasmus MC, University Medical Center Rotterdam, Rotterdam, the Netherlands

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María A Gálvez Moreno Maimonides Institute for Biomedical Research of Cordoba (IMIBIC); Reina Sofia University Hospital, Córdoba, Spain

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Justo P Castaño Maimonides Institute for Biomedical Research of Cordoba (IMIBIC); Reina Sofia University Hospital, Córdoba, Spain

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Wouter W de Herder Division of Endocrinology, Department of Internal Medicine, Erasmus MC, University Medical Center Rotterdam, Rotterdam, the Netherlands

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Richard A Feelders Division of Endocrinology, Department of Internal Medicine, Erasmus MC, University Medical Center Rotterdam, Rotterdam, the Netherlands

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original articles, were included. The following search terms, alone or in combination, were used: ‘biomarkers’, ‘novel markers’, ‘neuroendocrine tumors’, ‘NETs’, ‘neuroendocrine neoplasms’, ‘NENs’, ‘circulating markers’, ‘tissue markers’, ‘therapeutic

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Thomas Cuny Division Endocrinology, Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands
Aix-Marseille Université, Institut National de la Santé et de la Recherche Médicale (INSERM), U1251, Marseille Medical Genetics (MMG), Marseille, France
Department of Endocrinology, Assistance Publique – Hôpitaux de Marseille (AP-HM), Hôpital de la Conception, Centre de Référence des Maladies Rares Hypophysaires HYPO, Marseille, France

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Wouter de Herder Division Endocrinology, Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands

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Anne Barlier Aix-Marseille Université, Institut National de la Santé et de la Recherche Médicale (INSERM), U1251, Marseille Medical Genetics (MMG), Marseille, France
Department of Endocrinology, Assistance Publique – Hôpitaux de Marseille (AP-HM), Hôpital de la Conception, Centre de Référence des Maladies Rares Hypophysaires HYPO, Marseille, France

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Leo J Hofland Division Endocrinology, Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands

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distributions of small intestine neuroendocrine tumors (SI-NETs) and rectal NETs differ between region of the world, genders and ethnicities ( Fig. 1 ) ( Hauso et al. 2008 , Yao et al. 2008 , Ito et al. 2010 , Landerholm et al. 2010 , Taghavi et al

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Atsuko Kasajima Department of Pathology, Technical University Munich, Munich, Germany
Member of the German Cancer Consortium (DKTK), Germany

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Günter Klöppel Department of Pathology, Technical University Munich, Munich, Germany

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Introduction Neuroendocrine neoplasms (NENs) arise from various anatomical sites in the body but share basic features. NENs consist of two main families, well-differentiated NENs, also called neuroendocrine tumors (NETs) or carcinoids, and

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Tanupriya Contractor Raymond and Beverly Sackler Foundation, New Brunswick, New Jersey, USA

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Richard Clausen Raymond and Beverly Sackler Foundation, New Brunswick, New Jersey, USA

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Grant R Harris Raymond and Beverly Sackler Foundation, New Brunswick, New Jersey, USA

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Jeffrey A Rosenfeld Rutgers Cancer Institute of New Jersey, New Brunswick, New Jersey, USA

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Darren R Carpizo Rutgers Cancer Institute of New Jersey, New Brunswick, New Jersey, USA

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Laura Tang Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA

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Chris R Harris Raymond and Beverly Sackler Foundation, New Brunswick, New Jersey, USA
Rutgers Cancer Institute of New Jersey, New Brunswick, New Jersey, USA
Department of Surgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA

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Introduction Neuroendocrine tumors (NETs) can occur throughout the body, within the many organs that contain hormone-producing cells. Tumors found in these various locations can share common genetic drivers, as indicated by the fact that

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