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Zollinger-Ellison Syndrome
Atif Zaheer, MD; Michael P. Federle, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Zollinger-Ellison syndrome (ZES)
            • Definitions

              • Severe peptic ulcer disease associated with marked ↑ in gastric acid due to gastrin-producing neuroendocrine tumor (gastrinoma) of pancreas

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Angiographic Findings

                        • Nuclear Medicine Findings

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. Davila A et al: Multiple endocrine neoplasia: spectrum of abdominal manifestations. AJR Am J Roentgenol. 1-11, 2020
                                                2. Tiwari R et al: Radiologist's primer on imaging of common hereditary cancer syndromes. Radiographics. 39(3):759-78, 2019
                                                3. Epelboym I et al: Zollinger-Ellison syndrome: classical considerations and current controversies. Oncologist. 19(1):44-50, 2014
                                                4. Krampitz GW et al: Current management of the Zollinger-Ellison syndrome. Adv Surg. 47:59-79, 2013
                                                5. Metz DC et al: Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology. 135(5):1469-92, 2008
                                                6. Klose KJ et al: Localisation and staging of gastrin producing tumours using cross-sectional imaging modalities. Wien Klin Wochenschr. 119(19-20):588-92, 2007
                                                7. Levy AD et al: From the archives of the AFIP: Gastrointestinal carcinoids: imaging features with clinicopathologic comparison. Radiographics. 27(1):237-57, 2007
                                                8. Levy AD et al: Duodenal carcinoids: imaging features with clinical-pathologic comparison. Radiology. 237(3):967-72, 2005
                                                9. Pfannenberg AC et al: Dual-phase multidetector thin-section CT in detecting duodenal gastrinoma. Abdom Imaging. 30(5):543-7, 2005
                                                10. Nino-Murcia M et al: Multidetector-row CT and volumetric imaging of pancreatic neoplasms. Gastroenterol Clin North Am. 31(3):881-96, 2002
                                                11. Oshikawa O et al: Dynamic sonography of pancreatic tumors: comparison with dynamic CT. AJR Am J Roentgenol. 178(5):1133-7, 2002
                                                12. Rodallec M et al: Helical CT of pancreatic endocrine tumors. J Comput Assist Tomogr. 26(5):728-33, 2002
                                                13. Sheth S et al: Helical CT of islet cell tumors of the pancreas: typical and atypical manifestations. AJR Am J Roentgenol. 179(3):725-30, 2002
                                                14. Sheth S et al: Imaging of uncommon tumors of the pancreas. Radiol Clin North Am. 40(6):1273-87, vi, 2002
                                                15. Fidler JL et al: Imaging of neuroendocrine tumors of the pancreas. Int J Gastrointest Cancer. 30(1-2):73-85, 2001
                                                16. Ichikawa T et al: Islet cell tumor of the pancreas: biphasic CT versus MR imaging in tumor detection. Radiology. 216(1):163-71, 2000
                                                17. Buetow PC et al: Islet cell tumors of the pancreas: clinical, radiologic, and pathologic correlation in diagnosis and localization. Radiographics. 17(2):453-72; quiz 472A-B, 1997
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Top Differential Diagnoses

                                                      • Pathology

                                                        • Clinical Issues

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Zollinger-Ellison syndrome (ZES)
                                                          • Definitions

                                                            • Severe peptic ulcer disease associated with marked ↑ in gastric acid due to gastrin-producing neuroendocrine tumor (gastrinoma) of pancreas

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Ultrasonographic Findings

                                                                    • Angiographic Findings

                                                                      • Nuclear Medicine Findings

                                                                        • Imaging Recommendations

                                                                          DIFFERENTIAL DIAGNOSIS

                                                                            PATHOLOGY

                                                                            • General Features

                                                                              • Gross Pathologic & Surgical Features

                                                                                • Microscopic Features

                                                                                  CLINICAL ISSUES

                                                                                  • Presentation

                                                                                    • Demographics

                                                                                      • Natural History & Prognosis

                                                                                        • Treatment

                                                                                          DIAGNOSTIC CHECKLIST

                                                                                          • Consider

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. Davila A et al: Multiple endocrine neoplasia: spectrum of abdominal manifestations. AJR Am J Roentgenol. 1-11, 2020
                                                                                              2. Tiwari R et al: Radiologist's primer on imaging of common hereditary cancer syndromes. Radiographics. 39(3):759-78, 2019
                                                                                              3. Epelboym I et al: Zollinger-Ellison syndrome: classical considerations and current controversies. Oncologist. 19(1):44-50, 2014
                                                                                              4. Krampitz GW et al: Current management of the Zollinger-Ellison syndrome. Adv Surg. 47:59-79, 2013
                                                                                              5. Metz DC et al: Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology. 135(5):1469-92, 2008
                                                                                              6. Klose KJ et al: Localisation and staging of gastrin producing tumours using cross-sectional imaging modalities. Wien Klin Wochenschr. 119(19-20):588-92, 2007
                                                                                              7. Levy AD et al: From the archives of the AFIP: Gastrointestinal carcinoids: imaging features with clinicopathologic comparison. Radiographics. 27(1):237-57, 2007
                                                                                              8. Levy AD et al: Duodenal carcinoids: imaging features with clinical-pathologic comparison. Radiology. 237(3):967-72, 2005
                                                                                              9. Pfannenberg AC et al: Dual-phase multidetector thin-section CT in detecting duodenal gastrinoma. Abdom Imaging. 30(5):543-7, 2005
                                                                                              10. Nino-Murcia M et al: Multidetector-row CT and volumetric imaging of pancreatic neoplasms. Gastroenterol Clin North Am. 31(3):881-96, 2002
                                                                                              11. Oshikawa O et al: Dynamic sonography of pancreatic tumors: comparison with dynamic CT. AJR Am J Roentgenol. 178(5):1133-7, 2002
                                                                                              12. Rodallec M et al: Helical CT of pancreatic endocrine tumors. J Comput Assist Tomogr. 26(5):728-33, 2002
                                                                                              13. Sheth S et al: Helical CT of islet cell tumors of the pancreas: typical and atypical manifestations. AJR Am J Roentgenol. 179(3):725-30, 2002
                                                                                              14. Sheth S et al: Imaging of uncommon tumors of the pancreas. Radiol Clin North Am. 40(6):1273-87, vi, 2002
                                                                                              15. Fidler JL et al: Imaging of neuroendocrine tumors of the pancreas. Int J Gastrointest Cancer. 30(1-2):73-85, 2001
                                                                                              16. Ichikawa T et al: Islet cell tumor of the pancreas: biphasic CT versus MR imaging in tumor detection. Radiology. 216(1):163-71, 2000
                                                                                              17. Buetow PC et al: Islet cell tumors of the pancreas: clinical, radiologic, and pathologic correlation in diagnosis and localization. Radiographics. 17(2):453-72; quiz 472A-B, 1997