Journal Mobile Options
Table of Contents
Vol. 62, Suppl. 1, 2000
Issue release date: July 2000
Digestion 2000;62(suppl 1):51–58

Natural History of Neuroendocrine Enteropancreatic Tumors

Mignon M.
Department of Hepato-Gastroenterology and Inserm U.410, Paris, France

Individual Users: Register with Karger Login Information

Please create your User ID & Password

Contact Information

I have read the Karger Terms and Conditions and agree.

To view the fulltext, please log in

To view the pdf, please log in


Neuroendocrine enteropancreatic tumors (NEEPTs) include tumors developing from the pancreatic islets and the duodenal loop, and those developing from gut serotonin-secreting cells (mid-gut carcinoids). When functioning, they are rare tumors with frequently inconsistant clinical and biological characteristics. Provocative dynamic tests are very useful. Their natural history, with the exception of insulinomas and mid-gut carcinoids, is still incompletely known due to their rarity, relatively recent description, slow evolution and the absence of definitive reliable markers for prognosis. The following factors affect the natural history of NEEPTs. (1) Inclusion or not within the spectrum of multiple endocrine neoplasia type-1 (MEN1), such an integration often dramatically modifies the management strategy. In patients with MEN1, NEEPTs are often multiple, diffusely spread in the duodenopancreatic area and of small size. Definite cure by surgery is then very scarce. Mid-gut carcinoid tumors are the most protean NEEPTs. In contrast to appendiceal carcinoids, usually with a benign course, mid-gut carcinoids have a more malignant course and are very common indeed: local transmural invasion, early metastasis to lymph nodes and/or liver, and symptoms from hormone oversecretion. Carcinoid syndrome is usually well controlled by long-acting somatostatin analogs. (2) Disease spreading, i.e. liver and bone metastases, are the major causes of death in patients with NEEPTs. Liver metastases (LMs) are synchronous in about 75% of the patients with LMs but may develop during follow-up (about 25% of the patients with LMs were without LMs at the initial evaluation). Development of bone metastases has a very poor prognosis accompanying LM progression.

Copyright / Drug Dosage

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.


  1. Norton JA: Neuroendocrine tumors of the pancreas and duodenum. Curr Probl Surg 1994;31:81–156.
  2. Mignon M, Jensen RT (eds): Endocrine Tumors of the Pancreas. Recent Advances in Research and Management. Front Gastrointest Res Basel, Karger, 1995, vol 23, pp 1–482.
  3. Kaplan LM: Endocrine tumors of the gastrointestinal tract and pancreas; in Fauci AS, Brauwald E, Isselbacher KS, Wilson ID, Martin JB, Kasper DL, Hauser SL, Longo DL (eds): Harrison’s: Principles of Internal Medicine ed 14. New York, McGraw-Hill, 1999, pp 584–592.
  4. Delcore R, Friesen SR: Other rare tumors of the endocrine pancreas; in Howard J, Idezuki Y, Ihse I, Prinz R (eds): Surgical Diseases of the Pancreas. Baltimore, William & Wilkins, 1998, pp 789–815.
  5. Jensen RT, Norton JA: Endocrine tumors of the pancreas; in Feldman M, Scharschmidt BF, Sleisenger MH (eds): Gastrointestinal and Liver Disease, ed 6. Philadelphia, Saunders, 1998, vol 1, pp 871–894.
  6. Eriksson B, Oberg K, Skogseid B: Neuroendocrine pancreatic tumors. Clinical findings in a prospective study of 84 patients. Acta Oncol 1989;28:373–377.

    External Resources

  7. Mignon M, Cadiot G: Gastrinomas (Zollinger-Ellison syndrome); in Howard J, Idezuki Y, Ihse I, Prinz R (eds). Surgical Diseases of the Pancreas. Baltimore, Williams & Wilkins, 1997, pp 733–744.
  8. Cadiot G, Vuagnat A, Doukhan I, Murat A, Bonnaud G, Delemer B, Thiefin G, Beckers A, Veyrac M, Proye C, Ruszniewski Ph, Mignon M: Prognostic factors in patients with Zollinger-Ellison syndrome and multiple endocrine neoplasia type I. The experience of GENEM and GRESZE. Gastroenterology 1999;116:286–293.
  9. Jensen RT: Natural history of digestive endocrine tumors; in Mignon M, Colombel JF (eds): Recent Advances in the Pathophysiology and Management of Inflammatory Bowel Diseases and Digestive Endocrine Tumors. Paris, John Libbey Eurotext, 1999, pp 192–219.
  10. Kwekkeboom DJ, Krenning EP, Oei HY, Van Eyck CHJ, Lamberts SWJ: Use of radiolabeled somatostatin to localize islet cell tumors; in Mignon M, Jensen RT (eds): Endocrine Tumors of the Pancreas. Recent Advances in Research and Management. Front Gastrointest Res. Basel, Karger, 1995, vol 23, pp 298–308.
  11. Gibril F, Reynolds JC, Doppman JL, Chen CC, Termanini B, Stewart CA, Jensen RT: Does the use of octreoscanning alter management in patients with Zollinger-Ellison syndrome: a prospective study? Gastroenterology 1995;108:A194.
  12. Gibril F, Reynolds JC, Doppman JL, Chen CC, Venzon DJ, Termanini G: Somatostatin receptor scintigraphy: Its sensitivity compared with that of other imaging methods in detecting primary and metastatic gastrinomas. Ann Intern Med 1996;125:26–34.
  13. Lebtahi R, Cadiot G, Sarda L, Daou D, Faraggi M, Petegnief Y, Mignon M, Le Guludec D: Clinical impact of somatostatin receptor scintigraphy in the management of patients with neuroendocrine gastroenteropancreatic tumors. J Nucl Med 1997;38:853–858.
  14. Capella C, La Rosa S, Solcia E: Criteria for malignancy in pancreatic tumors. Endocr Pathol 1997;8:87–90.
  15. Zayène A, Cadiot G, Vuagnat A, Bonnaud G, Mignon M: Survival factors in patients with endocrine liver metastases. Gastroenterology 1997;112:A496.
  16. Sutliff VE, Doppman JL, Gibril F, Venzon DJ, Fang Y, Serrano J, Jensen RT: Growth of newly diagnosed untreated metastatic. Gastrinomas and predictors of growth pattern. J Clin Oncol 1997;15:2420–2431.
  17. Skogseid B, Eriksson B, Lundqvist G, Lörelius LE, Rastad J, Wide L, Wilander E, Akerström G, Oberg K: Multiple endocrine neoplasia type I: A ten-year prospective screening study in four kindreds. J Clin Endocrinol Metab 1991;73:281–287.

    External Resources

  18. Mignon M, Cadiot G, Rigaud D, Ruszniewski Ph, Jaïs P, Lehy T, Lewin MJM: Management of islet cell tumors in patients with multiple endocrine neoplasia type I; in Mignon M, Jensen RT (eds): Endocrine Tumors of the Pancreas. Recent Advances in Research and Management. Front Gastrointest Res. Basel, Karger, 1995;vol 23;pp 342–360.
  19. Mignon M, Ruszniewski Ph, Podevin Ph, Sabbagh L, Cadiot G, Rigaud D, Bonfils S: Current approach to the management of gastrinoma and insulinoma in adults with multiple endocrine neoplasia type I. World J Surg 1993;17:489–497.
  20. Chanson P, Cadiot G, Murat A, on behalf of GENEM 1: Management of patients and subjects at risk for multiple endocrine neoplasia type 1: MEN1. Horm Res 1997;47:211–220.
  21. Eriksson B, Arnberg H, Lindgren PG, Lörelius LE, Magnusson A, Lundqvist G, Skogseid B, Öberg K, Wide L, Wilander E: Neuroendocrine pancreatic tumors: Clinical presentation, biochemical and histopathological findings in 84 patients. J Intern Med 1990;228:103–113.
  22. Cadiot G, Houillier P, Allouch A, Paillard M, Mignon M, GRESZE: Interest of the oral calcium tolerance test in the early diagnosis of primary hyperparathyroidism and multiple endocrine neoplsia type I in patients with the Zollinger-Ellsion syndrome. Gut 1996;39:273–278.
  23. Cadiot G, Bonnaud G, Lebtahi R, Sarda L, Ruszniewski P, Le Guludec D, Mignon M, GRESZE. Usefulness of somatostatin receptor scintigraphy in the management of patients with Zollinger-Ellison syndrome. Gut 1997;41:107–114.
  24. Weber HC, Venzon DJ, Lin JT, Fishbein VA, Orbuch M, Strader DB, Gibril F, Metz DC, Fraker DL, Norton JA, Jensen RT: Determinants of metastatic rate and survival in patients with Zollinger-Ellison syndrome: A prospective long-term study. Gastroenterology 1995;108:1637–1649.
  25. Mignon M, Cadiot G: Natural history of gastrinoma: Lessons from the past. Ital J Gastroenterol Hepatol 1999;31(suppl 2):98–103.
  26. Madeira I, Terris B, Voss M, Denys A, Sauvanet A, Fléjou JF, Belghiti J, Ruszniewski Ph: Prognostic factors in patients with endocrine tumours of the duodenopancreatic area. Gut 1998;43:422–427.
  27. Howard TJ, Sawicki MP, Stabile BE, Watt PC, Passaro E Jr: Biologic behavior of sporadic gastrinoma located to the right and left of the superior mesenteric artery. Am J Surg 1993;165:101–106.
  28. Ellison EC: Forty-year appraisal of gatrinoma. Back to the future. Ann Surg 1995;222:511–524.
  29. Mignon M, Ruszniewski Ph, Hochlaf S, Sobhani I, Cadiot G: Legmann P, René E, Bonfils S, Rigaud D, Benhamou G: Do endocrine liver metastases have intrinsic evolutive features helping decision making for liver transplantation? Gastroenterology 1991;100:A290.
  30. Wiedenmann B, Jensen RT, Mignon M, Modlin I, Skogseid B, Doherty G, Öberg K: Preoperative diagnosis and surgical management of neuroendocrine gastroenteropancreatic tumors: General recommendations by a consensus workshop. World J Surg 1998;22:309–318.

Pay-per-View Options
Direct payment This item at the regular price: USD 38.00
Payment from account With a Karger Pay-per-View account (down payment USD 150) you profit from a special rate for this and other single items.
This item at the discounted price: USD 26.50