Pseudomyxoma peritonei--experience from a tertiary referral centre

Pseudomyxoma peritonei is a clinical diagnosis of massive abdominal swelling by a gelatinous material, produced usually from an ovarian or appendiceal primary. It is a rare entity that is usually histologically benign but behaves clinically in a malignant fashion with recurrent growth, although not demonstrating histological stromal invasion. The disease remains localized to the peritoneal cavity and the clinical course is one of repeated episodes of intestinal obstruction caused by extrinsic compression that seem only to be relieved by surgical debulking. Variable responses have been obtained with adjuvant chemo-, radio- and immunotherapy, but these isolated responses are unable to be reproduced and so there is no accepted adjuvant treatment for this disease.