A Multifocal Pattern of Neuroendocrine Neoplasms Along the Appendix: A Series of Six Cases

Int J Surg Pathol. 2019 Sep;27(6):613-618. doi: 10.1177/1066896919845048. Epub 2019 Apr 30.

Abstract

We present 6 cases with multifocal appendiceal neuroendocrine tumors, including their clinical and histopathological findings. To our knowledge, this is the first description of a multifocal pattern of a neuroendocrine neoplasm arising in the appendix. All patients presented in a setting requiring an acute appendectomy. The number of tumors ranged from 2 to 5. Histopathological examination revealed WHO (World Health Organization) grade 1 tumor in 3 patients and WHO grade 2 in the other 3 patients. The median duration of follow-up in these patients was 70 months (range = 6-192 months). No metastatic disease was observed. According to these findings, a multifocal pattern of neuroendocrine neoplasms along the appendix seems not to be a predictor for local advanced or metastatic disease.

Keywords: appendectomy; appendicitis; appendix vermiformis; neuroendocrine neoplasms.

MeSH terms

  • Adolescent
  • Adult
  • Appendectomy / methods
  • Appendiceal Neoplasms / pathology*
  • Appendiceal Neoplasms / surgery
  • Appendix / pathology*
  • Appendix / surgery
  • Female
  • Follow-Up Studies
  • Humans
  • Intestinal Neoplasms / pathology*
  • Intestinal Neoplasms / surgery
  • Laparoscopy / methods
  • Male
  • Neoplasm Grading
  • Neoplasms, Multiple Primary / pathology*
  • Neoplasms, Multiple Primary / surgery
  • Neuroendocrine Tumors / pathology*
  • Neuroendocrine Tumors / surgery
  • Pancreatic Neoplasms / pathology*
  • Pancreatic Neoplasms / surgery
  • Retrospective Studies
  • Stomach Neoplasms / pathology*
  • Stomach Neoplasms / surgery
  • Young Adult

Supplementary concepts

  • Gastro-enteropancreatic neuroendocrine tumor