[Peritoneal pseudomyxoma: an overview emphasizing pathological assessment and therapeutic strategies]

Ann Pathol. 2014 Feb;34(1):14-25. doi: 10.1016/j.annpat.2014.01.012. Epub 2014 Feb 25.
[Article in French]


Pseudomyxoma peritonei is a clinical entity characterized by a gelatinous ascite associated with mucinous tumor deposits spreading on peritoneal surface and potentially invading abdominal organs. It is considered as a tumor process linked, in most of cases, to a mucinous appendiceal neoplasm. Pseudomyxoma peritonei may benefit from a therapeutic strategy combining cytoreductive surgery and intra-peritoneal chemotherapy, which has led to a major prognosis improvement. Different classifications are available and the last one corresponds to the WHO 2010 version, which individualizes pseudomyxoma peritonei in two classes: low grade and high grade mucinous carcinoma. The very low frequency of this entity and its specific therapeutic strategy need specific health care centres, as well as physicians and pathologists collaborating through dedicated networks. The aim of this article is to summarize the pathology, causes, mechanisms and therapeutic approaches of pseudomyxoma peritonei, as well as their interfaces with dedicated networks.

Keywords: Appendice; Networks; Pseudomyxoma peritonei; Pseudomyxomes péritonéaux; Rares tumors; Réseaux; Tumeurs rares.

Publication types

  • English Abstract

MeSH terms

  • Humans
  • Peritoneal Neoplasms / classification
  • Peritoneal Neoplasms / pathology*
  • Peritoneal Neoplasms / therapy*
  • Pseudomyxoma Peritonei / classification
  • Pseudomyxoma Peritonei / pathology*
  • Pseudomyxoma Peritonei / therapy*