Disseminated peritoneal adenomucinosis: A critical review

Background: The term pseudomyxoma peritonei has been used in reference to a ny condition, benign or malignant, in which the peritoneal cavity becomes f illed with a gelatinous substance. The term is nonspecific and does not den ote therapeutic or prognostic significance. Controversy centers around whic h patients benefit from and should be treated by aggressive surgical debulk ing. Methods: A review of the current literature pertaining to the classificatio n, treatment, and prognosis of patients with pseudomyxoma peritonei was und ertaken. Results: Disseminated peritoneal adenomucinosis refers to a subset of patie nts with pseudomyxoma peritonei who derive the greatest long-term benefit f rom multimodality therapy including aggressive surgical debulking. These pa tients have a benign form of the disease in which the peritoneal implants a re derived from the extrusion of epithelial cells from an adenoma of the ap pendix. The pathophysiology of mucin deposition is defined by the redistrib ution phenomenon. The adenomatous cells are distributed according to the fl uid flow and gravitational forces within the peritoneal cavity. The small b owel is relatively spared until late in the disease (visceral sparing), and therefore aggressive surgical debulking should be attempted at the first l aparotomy by an experienced surgeon. Preoperative computed tomographic imag ing can establish the diagnosis and aid in defining which groups of patient s are resectable for cure. Conclusions: Attempts at curative treatment should include aggressive debul king and intraperitoneal chemotherapy. Those patients with a high-grade mal ignant process should be treated symptomatically, because aggressive therap y is associated with high morbidity rates and no long-term improvement in s urvival.