Skip to main content
SpringerLink
Log in

Gastrinoma – morphological aspects

Morphologische Aspekte des Gastrinoms

  • Published:
Wiener klinische Wochenschrift Aims and scope Submit manuscript

Zusammenfassung

Gastrinome sind Gastrin produzierende Tumore die durch einen erhöhten Nüchterngastrinspiegel, durch einen positiven Sekretintest und durch Symptome wie rezidivierende, peptische Ulzera oder Durchfälle (Zollinger-Ellison Syndrom) charakterisiert sind. Die meisten Gastrinome (rund 70%) haben ihren Sitz im Duodenum und nicht im Pankreas. Duodenalgastrinoma sind klein und können genetisch determiniert im Rahmen der multiplen endokrinen Neoplasie 1 (MEN1) vorkommen. Sie sind multizentrisch und entstehen aus Vorstufen. Die Prognose der Duodenalgastrinome ist trotz früher Lymphknotenmetastasierung besser als die der Pankreasgastrinome. Sie wachsen langsam und setzten spät Lebermetastasen.

Summary

Gastrinomas are defined as gastrin producing tumors that are associated with an elevated fasting gastrin serum level, a positive gastrin secretin stimulation test and certain clinical symptoms, e.g. recurrent peptic ulcer disease and occasionally diarrhea, the socalled Zollinger-Ellison syndrome. Most gastrinomas occur in the duodenum (approx. 70%) and not in the pancreas. The duodenal gastrinomas are small, and when they occur in association with the genetic syndrome of multiple endocrine neoplasia type 1 (MEN1), they are multicentric and originate from precursor lesions. The prognosis of duodenal gastrinomas is better than that of pancreatic gastrinomas, since despite early lymph node metastasis they progress slowly to liver metastasis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

References

  • Jensen RT, Gardner JD (1993) Gastrinoma. In: Go VLW, DiMagno EP, Gardner JD, Lebenthal E, Reber HA, Scheele GA (eds) The pancreas: biology, pathobiology and disease. Raven Press, New York, pp 931–978

    Google Scholar 

  • Zollinger RM (1987) Gastrinoma: the Zollinger-Ellison syndrome. Semin Oncol 14: 247–252

    PubMed  CAS  Google Scholar 

  • Zollinger RM, Ellison EH (1955) Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg 142: 709–723

    Article  PubMed  CAS  Google Scholar 

  • Gregory R, Tracy H (1959) The preparation and properties of gastrin. J Physiol (Lond) 149: 70–71

    Google Scholar 

  • Gregory RA, Tracy HJ, French JM, Sircus W (1960) Extraction of a gastrin-like substance from a pancreatic tumor in a case of Zollinger-Ellison syndrome. Lancet i: 1045–1048

    Article  Google Scholar 

  • Klöppel G, Willemer S, Stamm B, Häcki WH, Heitz PU (1986) Pancreatic lesions and hormonal profile of pancreatic tumors in multiple endocrine neoplasia type I. An immunocytochemical study of nine patients. Cancer 57: 1824–1832

    Article  PubMed  Google Scholar 

  • Solcia E, Capella C, Klöppel G (1997) Tumors of the pancreas. AFIP Atlas of Tumor Pathology, third series, fascicle 20. Armed Forces Institute of Pathology, Washington DC

    Google Scholar 

  • Ruszniewski P, Podevin P, Cadiot G, Marmuse JP, Mignon M, Vissuzaine C, Bonfils S, Lehy T (1993) Clinical, anatomical, and evolutive features of patients with the Zollinger-Ellison syndrome combined with type I multiple endocrine neoplasia. Pancreas 8: 295–304

    Article  PubMed  CAS  Google Scholar 

  • Polak JM, Stagg B, Pearse AG (1972) Two types of Zollinger-Ellison syndrome: immunofluorescent, cytochemical and ultrastructural studies of the antral and pancreatic gastrin cells in different clinical states. Gut 13: 501–512

    Article  PubMed  CAS  Google Scholar 

  • Friesen SR, Tomita T (1981) Pseudo-Zollinger-Ellison syndrome: hypergastrinemia, hyperchlorhydria without tumor. Ann Surg 194: 481–493

    Article  PubMed  CAS  Google Scholar 

  • Mehring UM, Jäger HJ, Klöppel G, Hasse FM (1997) Pankreatischer Polypeptid-sezernierender endokriner Pankreastumor assoziiert mit multiplen Ulcera ventriculi et duodeni. Langenbecks Arch Chir 382: 134–137

    PubMed  CAS  Google Scholar 

  • Chey WY, Chang TM, Lee KY, Sun G, Kim CK, You CH, Hamilton DL, Shah A, Rhee JC, Mutt V, et al (1989) Ulcerogenic tumor syndrome of the pancreas associated with a nongastrin acid secretagogue. Ann Surg 210: 139–149

    Article  PubMed  CAS  Google Scholar 

  • Donow C, Pipeleers-Marichal M, Schröder S, Stamm B, Heitz PU, Klöppel G (1991) Surgical pathology of gastrinoma. Site, size, multicentricity, association with multiple endocrine neoplasia type 1, and malignancy. Cancer 68: 1329–1334

    Article  PubMed  CAS  Google Scholar 

  • Oberhelman HA Jr, Nelsen TS (1964) Surgical consideration in the management of ulcerogenic tumors of the pancreas and duodenum. Am J Surg 108: 132–141

    Article  PubMed  Google Scholar 

  • Stamm B, Hedinger CE, Saremaslani P (1986) Duodenal and ampullary carcinoid tumors. A report of 12 cases with pathological characteristics, polypeptide content and relation to the MEN 1 syndrome and von Recklingshausen's disease (neurofibromatosis). Virchows Arch [A] Pathol Anat 408: 475–489

    Article  CAS  Google Scholar 

  • Thompson NW, Vinik AI, Eckhauser FE (1989) Microgastrinomas of the duodenum. A cause of failed operations for the Zollinger-Ellison syndrome. Ann Surg 209: 396–404

    Article  PubMed  CAS  Google Scholar 

  • Pipeleers-Marichal M, Donow C, Heitz PU, Klöppel G (1993) Pathologic aspects of gastrinomas in patients with Zollinger-Ellison syndrome with and without multiple endocrine neoplasia type I. World J Surg 17: 481–488

    Article  PubMed  CAS  Google Scholar 

  • Delcore R Jr, Cheung LY, Friesen SR (1988) Outcome of lymph node involvement in patients with the Zollinger-Ellison syndrome. Ann Surg 208: 291–298

    Article  PubMed  Google Scholar 

  • Wolfe MM, Alexander RW, McGuigan JE (1982) Extrapancreatic, extraintestinal gastrinoma: effective treatment by surgery. N Engl J Med 306: 1533–1536

    Article  PubMed  CAS  Google Scholar 

  • Weber HC, Venzon DJ, Lin JT, Fishbein VA, Orbuch M, Strader DB, Gibril F, Metz DC, Fraker DL, Norton JA, Jensen RT (1995) Determinants of metastatic rate and survival in patients with Zollinger-Ellison syndrome: a prospective long-term study. Gastroenterology 108: 1637–1649

    Article  PubMed  CAS  Google Scholar 

  • Thompson JC, Lewis BG, Wiener I, Townsend CM Jr (1983) The role of surgery in the Zollinger-Ellison syndrome. Ann Surg 197: 594–607

    PubMed  CAS  Google Scholar 

  • Heitz PU, Kasper M, Polak JM, Klöppel G (1982) Pancreatic endocrine tumours. Immunohistochemical analysis of 125 tumours. Hum Pathol 13: 263–271

    Article  PubMed  CAS  Google Scholar 

  • Stabile BE, Passaro E Jr (1985) Benign and malignant gastrinoma. Am J Surg 149: 144–150

    Article  PubMed  CAS  Google Scholar 

  • Schwarting H, Osse G, Sippel M, Arnold R, Creutzfeldt W (1983) Morphometry of the pancreatic islets in patients with insulinomas and gastrinomas. In: Mutt V, Uvnäs-Moberg K (eds) Regulatory peptides. Abstracts of the 4th international symposium on gastrointestinal hormones. Elsevier, Amsterdam, p 129

  • Martella EM, Ferraro G, Azzoni C, Marignani M, Bordi C (1997) Pancreatic-polypeptide cell hyperplasia associated with pancreatic or duodenal gastrinomas. Hum Pathol 28: 149–153

    Article  PubMed  CAS  Google Scholar 

  • Bordi C, Costa A, Missale G (1975) ECL cell proliferation and gastrin levels. Gastroenterology 68: 205–206

    PubMed  CAS  Google Scholar 

  • Solcia E, Bordi C, Creutzfeldt W, Dayal Y, Dayan AD, Falkmer S, Grimelius L, Havu N (1988) Histopathological classification of nonantral gastric endocrine growths in man. Digestion 41: 185–200

    Article  PubMed  CAS  Google Scholar 

  • D'Adda T, Corleto V, Pilato FP, Baggi MT, Robutti F, Delle Fave G, Bordi C (1990) Quantitative ultrastructure of endocrine cells of oxyntic mucosa in Zollinger-Ellison syndrome. Correspondence with light microscopic findings. Gastroenterology 99: 17–26

    PubMed  Google Scholar 

  • Solcia E, Capella C, Fiocca R, Rindi G, Rosai J (1990) Gastric argyrophil carcinoidosis in patients with Zollinger-Ellison syndrome due to type 1 multiple endocrine neoplasia. A newly recognized association. Am J Surg Pathol 14: 503–513

    PubMed  CAS  Google Scholar 

  • Pipeleers-Marichal M, Somers G, Willems G, Foulis A, Imrie C, Bishop AE, Polak JM, Häcki WH, Stamm B, Heitz PU, Klöppel G (1990) Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger-Ellison syndrome. N Engl J Med 322: 723–727

    Article  PubMed  CAS  Google Scholar 

  • Anlauf M, Perren A, Meyer CL, Schmid S, Saremaslani P, Kruse ML, Weihe E, Komminoth P, Heitz PU, Klöppel G (2005) Precursor lesions in patients with multiple endocrine neoplasia type 1-associated duodenal gastrinomas. Gastroenterology 128: 1187–1198

    Article  PubMed  CAS  Google Scholar 

  • Vella MA, Cowie AG, Gorsuch AN, Watson LC (1988) Giant gastrinoma in a patient with multiple endocrine adenopathy (type 1). J R Soc Med 81: 359–360

    PubMed  CAS  Google Scholar 

  • Anlauf M, Schlenger R, Perren A, Bauersfeld J, Koch CA, Dralle H, Raffel A, Knoefel WT, Weihe E, Ruszniewski P, Couvelard A, Komminoth P, Heitz PU, Klöppel G (2006) Microadenomatosis of the endocrine pancreas in patients with and without the multiple endocrine neoplasia type 1 syndrome. Am J Surg Pathol (in press)

  • Klöppel G, Clemens A (1996) The biological relevance of gastric neuroendocrine tumors. Yale J Biol Med 69: 69–74

    PubMed  Google Scholar 

  • Solcia E, Capella C, Buffa R, Grigerio G, Riocca R (1980) Pathology of the Zollinger-Ellison syndrome. In: Fenoglio LM, Wolf M (eds) Progress in surgical pathology. Masson, New York, pp 119–133

    Google Scholar 

  • Antonioli DA, Dayal Y, Dvorak AM, Banks PA (1987) Zollinger-Ellison syndrome. Cure by surgical resection of a jejunal gastrinoma containing growth hormone releasing factor. Gastroenterology 92: 814–823

    PubMed  CAS  Google Scholar 

  • Stabile BE, Morrow DJ, Passaro E Jr (1984) The gastrinoma triangle: operative implications. Am J Surg 147: 25–31

    Article  PubMed  CAS  Google Scholar 

  • Nord KS, Joshi V, Hanna M, Khademi M, Saad S, Marquis J, Pelzman H, Verner E (1986) Zollinger-Ellison syndrome associated with a renal gastrinoma in a child. J Pediatr Gastroenterol Nutr 5: 980–986

    Article  PubMed  CAS  Google Scholar 

  • Margolis RM, Jang N (1984) Zollinger-Ellison syndrome associated with pancreatic cystadenocarcinoma. N Engl J Med 311: 1380–1381

    PubMed  CAS  Google Scholar 

  • Morgan DR, Wells M, MacDonald RC, Johnston D (1985) Zollinger-Ellison syndrome due to a gastrin secreting ovarian mucinous cystadenoma. Case report. Br J Obstet Gynaecol 92: 867–869

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology, University of Kiel, Kiel, Germany

    Günter Klöppel & Martin Anlauf

Authors
  1. Günter Klöppel
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Martin Anlauf
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Günter Klöppel.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Klöppel, G., Anlauf, M. Gastrinoma – morphological aspects. Wien Klin Wochenschr 119, 579–584 (2007). https://doi.org/10.1007/s00508-007-0885-1

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00508-007-0885-1

Keywords

Search

Navigation