Incidentally found mucinous epithelial tumors of the appendix with or without pseudomyxoma peritonei: diagnostic and therapeutic algorithms based on current evidence

Acta Chir Belg. 2021 Aug;121(4):225-234. doi: 10.1080/00015458.2021.1894734. Epub 2021 May 31.


Mucinous appendiceal tumors with or without the pseudomyxoma peritonei (PMP) syndrome are rare, but often present as an incidental finding. The confusing histology and lack of large prospective trials result in a considerable diagnostic and therapeutic challenge in these patients. We propose treatment algorithms in patients with incidentally found mucinous epithelial appendiceal tumors, with or without PMP, based on the currently available evidence. The therapeutic approach should take into account the histology and grade of the primary appendix tumor, as well as those of the associated peritoneal disease.

Keywords: HAMN; HIPEC; LAMN; appendix; mucinous neoplasm.

MeSH terms

  • Algorithms
  • Appendiceal Neoplasms* / diagnosis
  • Appendiceal Neoplasms* / surgery
  • Humans
  • Peritoneal Neoplasms* / diagnosis
  • Peritoneal Neoplasms* / therapy
  • Prospective Studies
  • Pseudomyxoma Peritonei* / diagnosis
  • Pseudomyxoma Peritonei* / surgery