Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Neuroendocrine Tumor, Small Intestine

  • Ozgul SagolEmail author
Reference work entry


Carcinoid tumor; Endocrine neoplasm; Neuroendocrine carcinoma; Neuroendocrine neoplasm


Neuroendocrine neoplasm (NEN) is a general term including a broad family of neoplasms with neural and endocrine features. NENs may be localized in pure endocrine organs (tyroid, parathyroid and adrenal glands), nerve plexuses, extraadrenal paraganglia (paragangliomas) and diffuse endocrine system in different organs including gastrointestinal tract. Fourteen different cell types, producing variety of hormones have been described as components of diffuse endocrine system. Neuroendocrine cells of diffuse endocrine system that give rise to small intestinal NENs are endodermally derived and originate from gastrointestinal stem cells. Neuroendocrine cells and their neoplastic counterparts, secrete peptide hormones and biogenic amines. On electron microscopy, typical large neurosecretory granules and small synaptic like vesicles are found in their cytoplasm. These structures are also...

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References and Further Reading

  1. Capella, C., Arnold, R., Klimstra, D. S., Kloppel, G., Komminoth, P., Solcia, E., & Rind, G. (2010). Neuroendocrine neoplasms of the small intestine. In T. F. Bosman, F. Carneiro, R. H. Hruban, & N. D. Theise (Eds.), WHO classification of tumors of the digestive system (pp. 102–107). Lyon: IARC Press.Google Scholar
  2. Gianfranco, D. F., Kwekkeboom, D. J., Van Cutsem, E., Rindi, G., Kos-Kudla, B., Knigge, U., Sasano, H., Tomassetti, P., Salazar, R., Ruszniewski, P., & all other Barcelona Consensus Conference participants. (2012). ENETS consensus guidelines for the management of patients with gastroduodenal neoplasms. Neuroendocrinology, 95, 74–87.CrossRefGoogle Scholar
  3. Kloppel, G. (2011). Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms. Endocrine-Related Cancer, 18, S1–S16.CrossRefGoogle Scholar
  4. Kloppel, G., Rindi, G., Anlauf, M., Perren, A., & Komminoth, P. (2007). Site specific biology and pathology of gastroenteropancreatic neuroendocrine tumors. Virchows Archiv, 451(Suppl 1), S9–S27.CrossRefGoogle Scholar
  5. Niederle, B., Pape, U. F., Costa, F., Gross, D., Kelestimur, F., Knigge, U., Öberg, K., Pavel, M., Perren, A., Toumpanakis, C., O’Connor, J., O’Toole, D., Krenning, E., Reed, N., Kianmanesh, R., & all other Vienna Consensus Conference participants. (2016). ENETS consensus guidelines update for neuroendocrine neoplasm of the jejunum and ileum. Neuroendocrinology, 103, 125–138. 2 Jan 2016 [Epub ahead of print].CrossRefGoogle Scholar
  6. Rindi, G., & Wiedenmann, B. (2012). Neuroendocrine neoplasms of the gut and pancreas: New insights. Nature Reviews. Endocrinology, 8, 54–64.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Department of PathologyDokuz Eylul University Medical SchoolInciralti, IzmirTurkey