History of pseudomyxoma peritonei from its origin to the first decades of the twenty-first century

Abstract

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.