Recommendations in the management of epithelial appendiceal neoplasms and peritoneal dissemination from mucinous tumours (pseudomyxoma peritonei)

Clin Transl Oncol. 2016 May;18(5):437-48. doi: 10.1007/s12094-015-1413-9. Epub 2015 Oct 21.


The epithelial appendiceal neoplasms are uncommon and are usually detected as an unexpected surgical finding. The general surgeon should be aware of the diversity of its clinical manifestations and biological behaviors along with the significance of the surgical treatment on the progression of the illness and the prognosis of the patients. The operative findings and, especially, tumor histology, determine the type of surgery. Intestinal histologic subtype behaves and should be treated similarly to the right colon neoplasms; while mucinous tumors, often discordant between histology and its aggressiveness, can be treated with a simple appendectomy or require complex oncological surgeries. Mucinous tumors are often associated with the presence of mucin or tumor implants in the abdominal cavity, being the clinical syndrome known as pseudomyxoma peritonei (PMP). PMP tends to present an indolent but deadly evolution and requires a multimodal approach as a single treatment with curative potential: complete cytoreductive surgery plus hyperthermic Intra-peritoneal chemotherapy (CCRS + HIPEC) now considered the standard of care in this pathology. The general surgeon should be aware of the governing principles of the treatment of appendiceal neoplasms with or without peritoneal dissemination, know the therapeutic frontiers in every situation (avoiding unnecessary or counterproductive surgeries) and sending early these patients to specialised centres in the radical management of malignant diseases of the peritoneum in the conditions and with the necessary information to facilitate a possible radical treatment.

Keywords: Complete cytoreductive surgery (CCRS); Epithelial tumours of the appendix; Hyperthermic intra-peritoneal chemotheray (HIPEC); Mucinous appendiceal tumours; Mucinous dissemination from appendiceal neoplasm; Multimodality radical treatment of peritoneal carcinomatosis; Pseudomyxoma peritonei (PMP); Sugarbaker´s technique.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adenocarcinoma, Mucinous / pathology
  • Adenocarcinoma, Mucinous / therapy*
  • Appendiceal Neoplasms / secondary
  • Appendiceal Neoplasms / therapy*
  • Humans
  • Neoplasms, Glandular and Epithelial / secondary
  • Neoplasms, Glandular and Epithelial / therapy*
  • Peritoneal Neoplasms / secondary
  • Peritoneal Neoplasms / therapy*
  • Practice Guidelines as Topic*
  • Pseudomyxoma Peritonei