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. 1996 Jan-Feb;69(1):69–74.

The biological relevance of gastric neuroendocrine tumors.

G Klöppel 1, A Clemens 1
PMCID: PMC2588971  PMID: 9041691

Abstract

Gastric neuroendocrine tumors were originally thought to have a low incidence (three percent). Since endoscopic diagnostic procedures have become clinical routine, they are now found more frequently (relative incidence up to 41 percent). In recent years, classifications have been developed that attempt to consider the biological relevance of these tumors. Four types of gastric neuroendocrine tumor may be distinguished: Type 1 gastric neuroendocrine tumor is most common. It is associated with chronic atrophic fundus gastritis, hypergastrinemia and often with pernicious anemia. Usually it is multicentric and smaller than one cm, does not produce any symptoms and has an excellent prognosis. Type 2 gastric neuroendocrine tumor is second in frequency. It has no association with other diseases, is solitary and has no predilection for a particular localization. It may be larger than 1 cm, produce a carcinoid syndrome or Zollinger-Ellison syndrome and have a metastasis rate of up to 30 percent. Type 3 gastric neuroendocrine tumor is rare and always associated with Zollinger-Ellison syndrome and multiple endocrine neoplasia type I. It occurs as multiple lesions in the gastric body fundus and has a lower metastatic rate than type 2 gastric neuroendocrine tumor. Type 4 gastric neuroendocrine tumor corresponds to a small-cell carcinoma.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ahlman H., Dahlström A., Enerbäck L., Granérus G., Nilsson O., Persson S., Tisell L. E. Two cases of gastric carcinoids: diagnostic and therapeutic aspects. World J Surg. 1988 Jun;12(3):356–361. doi: 10.1007/BF01655671. [DOI] [PubMed] [Google Scholar]
  2. Ahlman H., Kölby L., Lundell L., Olbe L., Wängberg B., Granérus G., Grimelius L., Nilsson O. Clinical management of gastric carcinoid tumors. Digestion. 1994;55 (Suppl 3):77–85. doi: 10.1159/000201206. [DOI] [PubMed] [Google Scholar]
  3. Bordi C., Costa A., Missale G. Letter: ECL cell proliferation and gastrin levels. Gastroenterology. 1975 Jan;68(1):205–206. [PubMed] [Google Scholar]
  4. Bordi C., Pilato F. P., Bertelé A., D'Adda T., Missale G. Expression of glycoprotein hormone alpha-subunit by endocrine cells of the oxyntic mucosa is associated with hypergastrinemia. Hum Pathol. 1988 May;19(5):580–585. doi: 10.1016/s0046-8177(88)80208-9. [DOI] [PubMed] [Google Scholar]
  5. Bordi C., Yu J. Y., Baggi M. T., Davoli C., Pilato F. P., Baruzzi G., Gardini G., Zamboni G., Franzin G., Papotti M. Gastric carcinoids and their precursor lesions. A histologic and immunohistochemical study of 23 cases. Cancer. 1991 Feb 1;67(3):663–672. doi: 10.1002/1097-0142(19910201)67:3<663::aid-cncr2820670323>3.0.co;2-l. [DOI] [PubMed] [Google Scholar]
  6. Capella C., Heitz P. U., Höfler H., Solcia E., Klöppel G. Revised classification of neuroendocrine tumours of the lung, pancreas and gut. Virchows Arch. 1995;425(6):547–560. doi: 10.1007/BF00199342. [DOI] [PubMed] [Google Scholar]
  7. Chejfec G., Gould V. E. Malignant gastric neuroendogrinomas. Ultrastructural and biochemical characterization of their secretory activity. Hum Pathol. 1977 Jul;8(4):433–440. doi: 10.1016/s0046-8177(77)80007-5. [DOI] [PubMed] [Google Scholar]
  8. Chejfec G., Kovarick P., Graham G., Eichorst M., Gould V. E. Neuroendocrine carcinoma of the stomach with extensive somatostatin immunoreactivity. Ultrastruct Pathol. 1992 Sep-Oct;16(5):537–545. doi: 10.3109/01913129209061545. [DOI] [PubMed] [Google Scholar]
  9. Creutzfeldt W. The achlorhydria-carcinoid sequence: role of gastrin. Digestion. 1988;39(2):61–79. doi: 10.1159/000199609. [DOI] [PubMed] [Google Scholar]
  10. Feurle G. E. Argyrophil cell hyperplasia and a carcinoid tumour in the stomach of a patient with sporadic Zollinger-Ellison syndrome. Gut. 1994 Feb;35(2):275–277. doi: 10.1136/gut.35.2.275. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Godwin J. D., 2nd Carcinoid tumors. An analysis of 2,837 cases. Cancer. 1975 Aug;36(2):560–569. doi: 10.1002/1097-0142(197508)36:2<560::aid-cncr2820360235>3.0.co;2-4. [DOI] [PubMed] [Google Scholar]
  12. Hirschowitz B. I., Griffith J., Pellegrin D., Cummings O. W. Rapid regression of enterochromaffinlike cell gastric carcinoids in pernicious anemia after antrectomy. Gastroenterology. 1992 Apr;102(4 Pt 1):1409–1418. [PubMed] [Google Scholar]
  13. Håkanson R., Tielemans Y., Chen D., Andersson K., Ryberg B., Mattsson H., Sundler F. The biology and pathobiology of the ECL cells. Yale J Biol Med. 1992 Nov-Dec;65(6):761–829. [PMC free article] [PubMed] [Google Scholar]
  14. Kaplan E. L., Horvath K., Udekwu A., Straus F., 2nd, Schark C., Ferguson D. J., Skinner D. B. Gastrinomas: a 42-year experience. World J Surg. 1990 May-Jun;14(3):365–376. doi: 10.1007/BF01658530. [DOI] [PubMed] [Google Scholar]
  15. Larsson L. I., Ljungberg O., Sundler F., Håkanson R., Svensson S. O., Rehfeld J., Stadil R., Holst J. Antor-pyloric gastrinoma associated with pancreatic nesidioblastosis and proliferation of islets. Virchows Arch A Pathol Pathol Anat. 1973;360(4):305–314. doi: 10.1007/BF00548351. [DOI] [PubMed] [Google Scholar]
  16. MACDONALD R. A. A study of 356 carcinoids of the gastrointestinal tract; report of four new cases of the carcinoid syndrome. Am J Med. 1956 Dec;21(6):867–878. doi: 10.1016/0002-9343(56)90101-2. [DOI] [PubMed] [Google Scholar]
  17. Matsui K., Kitagawa M., Miwa A., Kuroda Y., Tsuji M. Small cell carcinoma of the stomach: a clinicopathologic study of 17 cases. Am J Gastroenterol. 1991 Sep;86(9):1167–1175. [PubMed] [Google Scholar]
  18. Mignon M., Ruszniewski P., Haffar S., Rigaud D., Rene E., Bonfils S. Current approach to the management of tumoral process in patients with gastrinoma. World J Surg. 1986 Aug;10(4):703–710. doi: 10.1007/BF01655562. [DOI] [PubMed] [Google Scholar]
  19. Mizuma K., Shibuya H., Totsuka M., Hayasaka H. Carcinoid of the stomach: a case report and review of 100 cases reported in Japan. Ann Chir Gynaecol. 1983;72(1):23–27. [PubMed] [Google Scholar]
  20. Murayama H., Imai T., Kikuchi M. Solid carcinomas of the stomach. A combined histochemical, light and electron microscopic study. Cancer. 1983 May 1;51(9):1673–1681. doi: 10.1002/1097-0142(19830501)51:9<1673::aid-cncr2820510920>3.0.co;2-q. [DOI] [PubMed] [Google Scholar]
  21. Norton J. A., Doppman J. L., Collen M. J., Harmon J. W., Maton P. N., Gardner J. D., Jensen R. T. Prospective study of gastrinoma localization and resection in patients with Zollinger-Ellison syndrome. Ann Surg. 1986 Oct;204(4):468–479. doi: 10.1097/00000658-198610000-00015. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Padberg B. C., Zornig C., Müller-Schwefe C., Klöppel G., Schröder S. Makrokarzinoidose des Magens bei einem MEN 1-Patienten mit Zollinger-Ellison-Syndrom und Hyperparathyreoidismus. Pathologe. 1992 Jul;13(4):215–220. [PubMed] [Google Scholar]
  23. Padberg B., Schröder S., Capella C., Frilling A., Klöppel G., Heitz P. U. Multiple endocrine neoplasia type 1 (MEN 1) revisited. Virchows Arch. 1995;426(6):541–548. doi: 10.1007/BF00192107. [DOI] [PubMed] [Google Scholar]
  24. Pipeleers-Marichal M., Somers G., Willems G., Foulis A., Imrie C., Bishop A. E., Polak J. M., Häcki W. H., Stamm B., Heitz P. U. Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger-Ellison syndrome. N Engl J Med. 1990 Mar 15;322(11):723–727. doi: 10.1056/NEJM199003153221103. [DOI] [PubMed] [Google Scholar]
  25. Rappel S., Altendorf-Hofmann A., Stolte M. Prognosis of gastric carcinoid tumours. Digestion. 1995;56(6):455–462. doi: 10.1159/000201276. [DOI] [PubMed] [Google Scholar]
  26. Rindi G., Luinetti O., Cornaggia M., Capella C., Solcia E. Three subtypes of gastric argyrophil carcinoid and the gastric neuroendocrine carcinoma: a clinicopathologic study. Gastroenterology. 1993 Apr;104(4):994–1006. doi: 10.1016/0016-5085(93)90266-f. [DOI] [PubMed] [Google Scholar]
  27. Roberts L. J., 2nd, Bloomgarden Z. T., Marney S. R., Jr, Rabin D., Oates J. A. Histamine release from a gastric carcinoid: provocation by pentagastrin and inhibition by somatostatin. Gastroenterology. 1983 Feb;84(2):272–275. [PubMed] [Google Scholar]
  28. Royston C. M., Brew D. S., Garnham J. R., Stagg B. H., Polak J. The Zollinger-Ellison syndrome due to an infiltrating tumour of the stomach. Gut. 1972 Aug;13(8):638–642. doi: 10.1136/gut.13.8.638. [DOI] [PMC free article] [PubMed] [Google Scholar]
  29. Russo A., Buffa R., Grasso G., Giannone G., Sanfilippo G., Sessa F., Solcia E. Gastric gastrinoma and diffuse G cell hyperplasia associated with chronic atrophic gastritis. Endoscopic detection and removal. Digestion. 1980;20(6):416–419. doi: 10.1159/000198484. [DOI] [PubMed] [Google Scholar]
  30. Sjöblom S. M., Sipponen P., Miettinen M., Karonen S. L., Jrvinen H. J. Gastroscopic screening for gastric carcinoids and carcinoma in pernicious anemia. Endoscopy. 1988 Mar;20(2):52–56. doi: 10.1055/s-2007-1018130. [DOI] [PubMed] [Google Scholar]
  31. Solcia E., Bordi C., Creutzfeldt W., Dayal Y., Dayan A. D., Falkmer S., Grimelius L., Havu N. Histopathological classification of nonantral gastric endocrine growths in man. Digestion. 1988;41(4):185–200. doi: 10.1159/000199786. [DOI] [PubMed] [Google Scholar]
  32. Solcia E., Capella C., Fiocca R., Rindi G., Rosai J. Gastric argyrophil carcinoidosis in patients with Zollinger-Ellison syndrome due to type 1 multiple endocrine neoplasia. A newly recognized association. Am J Surg Pathol. 1990 Jun;14(6):503–513. doi: 10.1097/00000478-199006000-00001. [DOI] [PubMed] [Google Scholar]
  33. Solt J., Kádas I., Polak J. M., Németh A., Bloom S. R., Rauth J., Horváth L. A pancreatic-polypeptide-producing tumor of the stomach. Cancer. 1984 Sep 15;54(6):1101–1104. doi: 10.1002/1097-0142(19840915)54:6<1101::aid-cncr2820540629>3.0.co;2-#. [DOI] [PubMed] [Google Scholar]
  34. Sweeney E. C., McDonnell L. M. Atypical gastric carcinoids. Histopathology. 1980 Mar;4(2):215–224. doi: 10.1111/j.1365-2559.1980.tb02914.x. [DOI] [PubMed] [Google Scholar]
  35. Thompson N. W., Vinik A. I., Eckhauser F. E., Strodel W. E. Extrapancreatic gastrinomas. Surgery. 1985 Dec;98(6):1113–1120. [PubMed] [Google Scholar]
  36. Tielemans Y., Håkanson R., Sundler F., Willems G. Proliferation of enterochromaffinlike cells in omeprazole-treated hypergastrinemic rats. Gastroenterology. 1989 Mar;96(3):723–729. [PubMed] [Google Scholar]
  37. Vogel S. B., Wolfe M. M., McGuigan J. E., Hawkins I. F., Jr, Howard R. J., Woodward E. R. Localization and resection of gastrinomas in Zollinger-Ellison syndrome. Ann Surg. 1987 May;205(5):550–556. doi: 10.1097/00000658-198705000-00014. [DOI] [PMC free article] [PubMed] [Google Scholar]

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