Pathology of the Gastrointestinal Tract

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

Appendiceal Tumors

  • Magali SvrcekEmail author
Reference work entry


Adenocarcinoma; Carcinoid; Crypt cell carcinoma; Goblet cell tumor; Goblet cell-type adenocarcinoid; High-grade neuroendocrine carcinoma; Microglandular carcinoma; Mucinous carcinoid tumor; Neuroendocrine neoplasm; Non-mucinous carcinoma; Poorly differentiated endocrine carcinoma; Signet ring cell carcinoma; Small cell and large cell endocrine carcinoma; Well-differentiated endocrine tumor/carcinoma


One of the particularities of the appendix is the higher incidence of neuroendocrine neoplasms than carcinomas. Neuroendocrine neoplasms (NENs) of the appendix correspond to neoplasms with neuroendocrine differentiation and include neuroendocrine tumors (NET), neuroendocrine carcinoma (NEC), and mixed adenoneuroendocrine carcinomas (MANEC). In the latter, both the endocrine and exocrine components exceed 30%.

Primary adenocarcinomas of the appendix are rare after one eliminates low-grade appendiceal mucinous neoplasms (LAMNs) and mucinous adenocarcinomas. In the current...

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References and Further Reading

  1. Deschamps, L., & Couvelard, A. (2010). Endocrine tumors of the appendix. Archives of Pathology & Laboratory Medicine, 134, 871–875.Google Scholar
  2. Edge, S. B., Byrd, D. R., Compton, C. C., Fritz, A. G., Greene, F. L., & Trotti, A. (2010). Appendix. In AJCC cancer staging handbook, from the AJCC cancer staging manual (7th ed., pp. 161–171). New York: Springer.Google Scholar
  3. Komminoth, P., Arnold, R., Capella, C., Klimstra, D.S., Kloppel, G., Solcia, E., & Rindi, G. (2010). Neuroendocine neoplasms of the appendix. In F. T. Bosman, F. Carneiro, R. H. Hruban, & N. D. Theise (Eds.), WHO classification of tumours of the digestive system (pp. 126–128). Lyon: IARC Press.Google Scholar
  4. Pape, U.-F., Perren, A., Niederle, B., et al. (2012). ENETS consensus guidelines for the management of patients with neuroendocrine neoplasms from the jejuno-ileum and the appendix including goblet cell carcinomas. Neuroendocrinology, 95, 135–156.PubMedGoogle Scholar
  5. Rindi, G., Arnold, R., Bosman, F. T., & Capella, C. (2010). Nomenclature and classification of neuroendocrine neoplasms of the digestive system. In F. T. Bosman, F. Carneiro, R. H. Hruban, & N. D. Theise (Eds.), WHO classification of tumours of the digestive system (pp. 13–14). Lyon: IARC Press.Google Scholar
  6. Volante, M., Daniele, L., & Asioli, S. (2013). Tumor staging but not grading is associated with adverse clinical outcome in neuroendocrine tumors of the appendix. A retrospective clinical pathologic analysis of 138 cases. The American Journal of Surgical Pathology, 37, 606–612.PubMedGoogle Scholar
  7. Yao, J. C., Hassan, M., Phan, A., et al. (2008). One hundred years after “carcinoid”: Epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. Journal of Clinical Oncology, 26, 3063–3072.PubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Hôpital Saint-Antoine, Service d’Anatomie Pathologique, AP-HPHôpitaux Universitaires de l’Est ParisienParisFrance