Disseminated peritoneal adenomucinosis: A critical review

Background: The term pseudomyxoma peritonei has been used in reference to any condition, benign or malignant, in which the peritoneal cavity becomes filled with a gelatinous substance. The term is nonspecific and does not denote therapeutic or prognostic significance. Controversy centers around which patients benefit from and should be treated by aggressive surgical debulking. Methods: A review of the current literature pertaining to the classification, treatment, and prognosis of patients with pseudomyxoma peritonei was undertaken. Results: Disseminated peritoneal adenomucinosis refers to a subset of patients with pseudomyxoma peritonei who derive the greatest long-term benefit from multimodality therapy including aggressive surgical debulking. These patients have a benign form of the disease in which the peritoneal implants are derived from the extrusion of epithelial cells from an adenoma of the appendix. The pathophysiology of mucin deposition is defined by the redistribution phenomenon. The adenomatous cells are distributed according to the fluid flow and gravitational forces within the peritoneal cavity. The small bowel is relatively spared until late in the disease (visceral sparing), and therefore aggressive surgical debulking should be attempted at the first laparotomy by an experienced surgeon. Preoperative computed tomographic imaging can establish the diagnosis and aid in defining which groups of patients are resectable for cure. Conclusions: Attempts at curative treatment should include aggressive debulking and intraperitoneal chemotherapy. Those patients with a high-grade malignant process should be treated symptomatically, because aggressive therapy is associated with high morbidity rates and no long-term improvement in survival.