Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Surg. Sep 27, 2019; 11(9): 358-364
Published online Sep 27, 2019. doi: 10.4240/wjgs.v11.i9.358
History of pseudomyxoma peritonei from its origin to the first decades of the twenty-first century
Francisco J Morera-Ocon, Clara Navarro-Campoy
Francisco J Morera-Ocon, Department of General Surgery, Hospital General de Requena, Valencia 46340, Spain
Clara Navarro-Campoy, Department of Gynecology and Obstetrics, Hospital 9 Octubre, Valencia 46340, Spain
Conflict-of-interest statement: The authors declare no potential conflicts of interest relevant to this article.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Francisco J Morera-Ocon, MD, PhD, Staff Physician, Surgeon, Surgical Oncologist, Department of General Surgery, Hospital General de Requena, Paraje Casablanca s/n, Valencia 46340, Spain.
Telephone: +34-96-2339180 Fax: +34-962304523
Received: May 2, 2019
Peer-review started: May 9, 2019
First decision: June 12, 2019
Revised: July 8, 2019
Accepted: August 7, 2019
Article in press: August 7, 2019
Published online: September 27, 2019

Pseudomyxoma peritonei (PMP) is a disease surrounded by misunderstanding and controversies. Knowledge about the etymology of pseudomyxoma is useful to remove the ambiguity around that term. The word pseudomyxoma derives from pseudomucin, a type of mucin. PMP was first described in a case of a woman alleged to have a ruptured pseudomucinous cystadenoma of the ovary, a term that has disappeared from today’s classifications of cystic ovarian neoplasms. It is known today that in the majority of cases, the origin for PMP is an appendiceal neoplasm, often of low histological grade. Currently, ovarian tumors are wrongly being considered a significant recognized etiology of PMP. PMP classification continues to be under discussion, and experts’ panels strive for consensus. Malignancy is also under discussion, and it is shown in this review that there is a long-standing historical reason for that. Surgery is the main tool in the treatment armamentarium for PMP, and the only therapy with potential curative option.

Keywords: Pseudomyxoma peritonei, Pseudomucin, Appendiceal neoplasm, Hyperthermic intraperitoneal chemotherapy

Core tip: Pseudomyxoma peritonei is an orphan disease that explains the misunderstanding around this nosologic entity. There is still controversy over its definition and classification. Cystadenocarcinoma of the ovary has been repeatedly and wrongly stated as an important etiology of pseudomyxoma. The aim of the present review is to provide clarifications on misconceptions surrounding PMP and to explain the historical sources from which such misconceptions have been drawn.