Pseudomyxoma peritonei

BACKGROUND: Pseudomyxoma peritonei is an unusual condition in which gelatinous fluid collections are associated with mucinous implants on the peritoneal surfaces and omentum. The pathological origin and ideal treatment of the condition are subjects of debate. METHODS: An unrestricted Medline search over 1986-1997 was performed for pseudomyxoma peritonei. RESULTS AND CONCLUSIONS: There is increasing evidence that pseudomyxoma peritonei is a neoplastic condition which usually arises from a primary adenoma or adenocarcinoma of the appendix. Reported series include a spectrum of pathological lesions, from entirely benign ruptured mucocele to advanced carcinoma. This, and the rarity of the condition, limit the conclusions that can be drawn regarding its treatment and prognosis. Most authorities agree that a thorough surgical debulking should be made. In most cases this will be a difficult and time-consuming undertaking, possibly requiring cooperation between two or more specialists and consideration of delivering intraperitoneal adjuvant therapy during or immediately after surgery. Treatment therefore requires a planned approach with accurate preoperative assessment of the diagnosis and the extent of the condition. There is some largely anecdotal evidence in favour of intraperitoneal chemotherapy and radioisotope treatment. Ultraradical surgery, with heated intraoperative and further postoperative chemotherapy, is strongly advocated by one group but remains contentious. The majority of patients will eventually suffer recurrence. The 5-year survival rate ranges from 53 to 75 per cent, but outcomes vary widely between relatively benign and malignant subgroups.