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Tumor Detection in Syndromic NET: Zollinger-Ellison Syndrome

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Neuroendocrine Tumors in Real Life

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Abstract

The Zollinger-Ellison syndrome (ZES) is a clinical disorder characterized by recurrent peptic ulcers due to hypergastrinemia induced by a gastrinoma, a gastrin-secreting neuroendocrine tumor. ZES is sporadic in 60–75% of cases, whereas in the remaining patients, it is associated with multiple endocrine neoplasia type 1 (MEN1), an autosomal dominant disorder resulting in hyperplasia and/or tumors of endocrine and non-endocrine organs. Here we present a case of a 40-year-old female, presented with recurrent epigastric pain and acid reflux, uncontrolled with proton pump inhibitors. A MEN1-related ZES was hypothesized on the basis of early age of onset and the evidence of multifocal tumors within the pancreas and duodenum, despite the absence of primary hyperparathyroidism. The diagnostic workup included endoscopic ultrasound, contrast-enhanced multi-detector computed tomography, and somatostatin-receptor scintigraphy.

On behalf of the ENETS Center of Excellence Multidisciplinary Group for Neuroendocrine Tumors in Naples, Italy

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Authors and Affiliations

  1. Enets Center of Excellence for Neuroendocrine Tumours, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy

    Roberta Modica & Annamaria Colao

  2. Enets Center of Excellence for Neuroendocrine Tumours, Department of Advanced Biomedical Sciences, Section of Diagnostic Imaging, University of Naples Federico II, Naples, Italy

    Luigi Camera

  3. Enets Center of Excellence for Neuroendocrine Tumours, Department of Cardiothoracic and Respiratory Sciences, Surgical Endoscopy Unit, University of Campania Luigi Vanvitelli, Naples, Italy

    Vincenzo Napolitano & Manuela Avellino

  4. Enets Center of Excellence for Neuroendocrine Tumours, Institute of Biostructures and Bioimaging, National Research Council, Naples, Italy

    Rosa Fonti

  5. Enets Center of Excellence for Neuroendocrine Tumours, Department of Advanced Biomedical Sciences, University of Naples Federico II, Naples, Italy

    Silvana Del Vecchio

  6. Enets Center of Excellence for Neuroendocrine Tumours, Gastroenterology and Digestive Endoscopy Unit, Pellegrini Hospital, Naples, Italy

    Leonardo De Luca

  7. Enets Center of Excellence for Neuroendocrine Tumours, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy

    Antongiulio Faggiano

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  1. Roberta Modica
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  2. Luigi Camera
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  4. Manuela Avellino
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  7. Leonardo De Luca
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  8. Annamaria Colao
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  9. Antongiulio Faggiano
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Correspondence to Roberta Modica .

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Editors and Affiliations

  1. ENETs Center of Excellence for Neuroendocrine Tumours, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy

    Annamaria Colao

  2. ENETs Center of Excellence for Neuroendocrine Tumours, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy

    Antongiulio Faggiano

  3. Endocrine Oncology, Internal Medicine Department, Erasmus University, Rotterdam, Zuid-Holland, The Netherlands

    Wouter de Herder

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Modica, R. et al. (2018). Tumor Detection in Syndromic NET: Zollinger-Ellison Syndrome. In: Colao, A., Faggiano, A., de Herder, W. (eds) Neuroendocrine Tumors in Real Life. Springer, Cham. https://doi.org/10.1007/978-3-319-59024-0_11

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  • DOI: https://doi.org/10.1007/978-3-319-59024-0_11

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-59022-6

  • Online ISBN: 978-3-319-59024-0

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