Neuroendocrine Neoplasms of the Appendix: A Review of the Literature.

Journal Article (Journal Article;Review)

Appendiceal neuroendocrine neoplasms (ANENs) comprise rare tumors of the appendix, mainly affecting young populations and characterized by a rather favorable prognosis. The aim of this review was to summarize the current knowledge on these neoplasms, focusing on the management and follow-up of such patients, which still remain under debate. ANENs account for 0.16-2.3% of appendectomies and are usually diagnosed incidentally. The histopathological diagnosis includes the immunohistochemical profile of the tumor in regard to synaptophysin and chromogranin A, as well as the Ki-67 index. The surgical management of ANENs is either simple appendectomy or a more extensive oncological operation including right hemicolectomy. This depends on the stage and the presence of risk factors suggesting a more aggressive disease, such as the exact location, mesoappendiceal or lymphovascular invasion, and the proliferative rate of the tumor. Despite their indolent course, ANENs may relapse. Therefore, lifetime observation is necessary for patients with tumors >2 cm and >1 cm plus additional risk factors; however, more studies should be conducted in order to determine the optimal follow-up strategy.

Full Text

Duke Authors

Cited Authors

  • Moris, D; Tsilimigras, DI; Vagios, S; Ntanasis-Stathopoulos, I; Karachaliou, G-S; Papalampros, A; Alexandrou, A; Blazer, DG; Felekouras, E

Published Date

  • February 2018

Published In

Volume / Issue

  • 38 / 2

Start / End Page

  • 601 - 611

PubMed ID

  • 29374682

Electronic International Standard Serial Number (EISSN)

  • 1791-7530

Digital Object Identifier (DOI)

  • 10.21873/anticanres.12264


  • eng

Conference Location

  • Greece