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: Appendiceal neoplasm; Hyperthermic intraperitoneal chemotherapy; Pseudomucin; Pseudomyxoma peritonei.

Figure 1

A 37-year-old woman operated on for a ruptured giant mucinous cystadenoma of the pancreas. A: Computed tomography image showing free mucinous ascites (arrows) and the dome of the pancreatic cystic lesion (mucinous cystadenoma with focal adenocarcinoma) in the retrogastric area; B: Peritoneal fluid in the right paracolic gutter (arrow) and corporal cystic lesion with septa.

Figure 2

Thoracic computed tomography images showing bilateral nodular metastases (arrows) from a low-grade appendiceal mucinous neoplasm.

Figure 3

Two forms of omental cake in two patients with peritoneal dissemination of low-grade appendiceal mucinous neoplasm. Note the sparing of the small bowel in the right picture.