Management of Appendix Neuroendocrine Neoplasms: Insights on the Current Guidelines

Amr Mohamed, Sulin Wu, Mohamed Hamid, Amit Mahipal, Sakti Cjakrabarti, David Bajor, J. Eva Selfridge, Sylvia L. Asa

Research output: Contribution to journalReview articlepeer-review


Appendiceal neuroendocrine neoplasms (ANENs) usually present as incidental findings at the time of appendectomy for acute appendicitis. They are rare, accounting for only 0.5–1% of intestinal neoplasms; they are found in 0.3–0.9% of all appendectomy specimens. They are usually sporadic tumors. There are several histological types including well-differentiated neuroendocrine tumors (NETs), poorly differentiated neuroendocrine carcinomas (NECs), and mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs). Histologic differentiation and the grade of well-differentiated NETs correlate with clinical behavior and prognosis. Management varies based on differentiation, aggressiveness, and metastatic potential. There is debate about the optimal surgical management for localized appendiceal NETs that are impacted by many factors including the tumor size, the extent of mesoappendiceal spread, lymphovascular invasion and perineural involvement. In addition, the data to guide therapy in metastatic disease are limited due to the paucity of these tumors. Here, we review the current advances in the management of ANENs within the context of a multidisciplinary approach to these tumors.

Original languageEnglish (US)
Article number295
Issue number1
StatePublished - Jan 2023


  • appendix
  • management
  • neoplasms
  • neuroendocrine

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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