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Andrea Weckman Division of Neurosurgery, Division of Pathology, Department of Neurosurgery, Department of Surgery

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Fabio Rotondo Division of Neurosurgery, Division of Pathology, Department of Neurosurgery, Department of Surgery

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Antonio Di Ieva Division of Neurosurgery, Division of Pathology, Department of Neurosurgery, Department of Surgery

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Luis V Syro Division of Neurosurgery, Division of Pathology, Department of Neurosurgery, Department of Surgery

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Henriett Butz Division of Neurosurgery, Division of Pathology, Department of Neurosurgery, Department of Surgery

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Michael D Cusimano Division of Neurosurgery, Division of Pathology, Department of Neurosurgery, Department of Surgery

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Kalman Kovacs Division of Neurosurgery, Division of Pathology, Department of Neurosurgery, Department of Surgery

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neoplasia, endocrine tumors occur more rarely ( Table 1 ). Endocrine tumors are defined as neoplasia of the hormone-secreting cells of the classic endocrine glands, including the pituitary, thyroid, parathyroid and adrenal glands, as well as the ovaries and

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Tomoko Sekiya Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Marcello D Bronstein Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Katiuscia Benfini Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Viviane C Longuini Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Raquel S Jallad Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Marcio C Machado Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Tatiana D Goncalves Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Luciana H Osaki Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Leonardo Higashi Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Jose Viana-Jr Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil
Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Claudio Kater Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Misu Lee Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Sara Molatore Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Guilherme Francisco Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Roger Chammas Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Michel S Naslavsky Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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David Schlesinger Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil
Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Patricia Gama Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Yeda A O Duarte Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Maria Lucia Lebrão Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Mayana Zatz Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Osorio Meirelles Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Bernardo Liberman Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Maria Candida B V Fragoso Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Sergio P A Toledo Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Natalia S Pellegata Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Rodrigo A Toledo Endocrine Genetics Unit LIM-25, Neuroendocrinology Unit, Adrenal Unit (LIM-42), Experimental Oncology Laboratory (LIM-24), Nursing School, School of Public Health, Endocrinology Division, Brigadeiro Hospital, Federal University of Sao Paulo, Human Genome Research Center, Department of Cell and Developmental Biology, Instituto do Cérebro, National Institute of Aging, Institute of Pathology, School of Medicine, Hospital das Clinicas, University of Sao Paulo, Sao Paulo, Brazil

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Introduction In the last two decades, genetic studies of endocrine tumor syndromes have identified a number of susceptibility genes including several tumor suppressors (multiple endocrine neoplasia type 1 ( MEN1 ), VHL , NF1 , SDHA , SDHB

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Michael Solarski Division of Neurosurgery, Department of Surgery, St. Michael’s Hospital, Toronto, Ontario, Canada

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Fabio Rotondo Division of Pathology, Department of Laboratory Medicine, St. Michael’s Hospital, Toronto, Ontario, Canada

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William D Foulkes Department of Human Genetics, Medicine and Oncology, McGill University, Montreal, Quebec, Canada
Lady Davis Institute, Jewish General Hospital and Research Institute, McGill University Health Centre, Montreal, Quebec, Canada

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John R Priest Department of Medicine, Minneapolis, Minnesota, USA

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Luis V Syro Department of Neurosurgery, Hospital Pablo Tobon Uribe and Clinica Medellin, Medellin, Colombia

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Henriett Butz Molecular Medicine Research Group, Hungarian Academy of Sciences, Semmelweis University, Budapest, Hungary

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Michael D Cusimano Division of Neurosurgery, Department of Surgery, St. Michael’s Hospital, Toronto, Ontario, Canada

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Kalman Kovacs Division of Pathology, Department of Laboratory Medicine, St. Michael’s Hospital, Toronto, Ontario, Canada

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well ( Fig. 1 ). Figure 1 Schematic diagram of the various locations of endocrine tumors associated with DICER1 syndrome. A full colour version of this figure is available at https://doi.org/10.1530/ERC-17-0509 . DICER1 is located on

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A Perren
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S Schmid
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T Locher
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P Saremaslani
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P U Heitz
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P Komminoth
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endocrine tumors have been conducted (with the exception of thyroid tumors), although there are several indications pointing towards a possible role of BRAF in this type of tumor also. First, like melanomas ( Dupin & Le Douarin 2003 ), a subset of endocrine

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Thorvardur R Halfdanarson
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Joseph Rubin
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Michael B Farnell Division of Oncology, Department of Surgery, Department of Health Sciences Research, Department of Medical Oncology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA

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Clive S Grant Division of Oncology, Department of Surgery, Department of Health Sciences Research, Department of Medical Oncology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA

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Gloria M Petersen Division of Oncology, Department of Surgery, Department of Health Sciences Research, Department of Medical Oncology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA

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Introduction Pancreatic endocrine tumors (PETs) are uncommon neoplasms with an incidence of <1 per 100 000 person-years in population studies ( Moldow & Connelly 1968 , Buchanan et al . 1986 , Eriksson et al . 1989 , Watson et al . 1989

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D Campana Dipartimento Clinico di Scienze Radiologiche ed Istopatologiche, Dipartimento di Medicina Interna e Gastroenterologia and

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F Nori Dipartimento Clinico di Scienze Radiologiche ed Istopatologiche, Dipartimento di Medicina Interna e Gastroenterologia and

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R Pezzilli Dipartimento Clinico di Scienze Radiologiche ed Istopatologiche, Dipartimento di Medicina Interna e Gastroenterologia and

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L Piscitelli Dipartimento Clinico di Scienze Radiologiche ed Istopatologiche, Dipartimento di Medicina Interna e Gastroenterologia and

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D Santini Dipartimento Clinico di Scienze Radiologiche ed Istopatologiche, Dipartimento di Medicina Interna e Gastroenterologia and

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E Brocchi Dipartimento Clinico di Scienze Radiologiche ed Istopatologiche, Dipartimento di Medicina Interna e Gastroenterologia and

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R Corinaldesi Dipartimento Clinico di Scienze Radiologiche ed Istopatologiche, Dipartimento di Medicina Interna e Gastroenterologia and

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P Tomassetti Dipartimento Clinico di Scienze Radiologiche ed Istopatologiche, Dipartimento di Medicina Interna e Gastroenterologia and

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exerts a trophic effect on ECL-cells, which leads to hyperplasia and, in some cases, to gastric endocrine tumors ( Waldum et al . 1998 , Lehy et al . 2000 ). In particular, type I tumors occur mostly in women and are rarely symptomatic ( Borch et al

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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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more common cancers, endocrine tumors sometimes do not attract equal attention and resources largely due to their relative rare nature. Fortunately for these patients, many endocrine cancer types have been investigated as part this genomic revolution

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Cuiqi Zhou Pathology, Departments of

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Yunguang Tong Pathology, Departments of

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Kolja Wawrowsky Pathology, Departments of

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Serguei Bannykh Pathology, Departments of

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Ines Donangelo Pathology, Departments of

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Shlomo Melmed Pathology, Departments of

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-time PCR (A and B); Oct-1 and hPTTG1 protein levels were measured by western blot (C and D). Oct-1 and hPTTG1 are concordantly expressed in endocrine tumors and colon cancers hPTTG1 is overexpressed in multiple human tumors including pituitary, breast, and

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Francesco Panzuto
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Silvia Nasoni
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Massimo Falconi
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Vito Domenico Corleto
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Gabriele Capurso
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Stefano Angeletti
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Maria Sofia Cattaruzza
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Gianfranco Delle Fave
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Introduction Gastro-entero-pancreatic endocrine tumors (GEP ETs) are rare neoplasms, with 1–4 cases per 100 000 people per year ( Quaedvlieg et al. 2001 , Modlin et al. 2003 , Lepage et al. 2004 , Taal & Visser 2004

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Yasuhiko Nagano
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Do Ha Kim
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Li Zhang
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Jill A White
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James C Yao
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Stanley R Hamilton
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Asif Rashid
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Introduction Pancreatic endocrine tumors (PETs) are uncommon, mostly well-differentiated and indolent neuroendocrine neoplasms, with an age-adjusted annual incidence of <1 per 100 000 ( Buchanan et al. 1986 ). The molecular

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