Management of pseudomyxoma peritonei

Pseudomyxoma peritonei (PMP) is a rare disease. It refers to a progressive disease process within the peritoneum which originates from the appendix or ovaries and is characterised by the production of copious amounts of mucinous fluid resulting in a "jelly belly". If untreated the condition is fatal. The traditional approach to PMP is based on repeated surgical debulking procedures, often associated with intraperitoneal or systemic chemotherapy. The natural history of this disease has been drastically modified since the introduction of a new surgical approach defined as a peritonectomy procedure. This paper is to review the literature on this treatment strategy.