Pseudomyxoma peritonei: a rare disease and a disease model in peritoneal surface oncology

Abstract

Peritoneal dissemination of abdominal and pelvic-based malignancies has attracted the clinical and scientifi c interest of surgical oncologists in the last two decades. Unprecedented survival results have been reported by the knowledgeable use of cytoreductive surgery combined with perioperative intraperitoneal chemotherapy and systemic chemotherapy in patients suffering from peritoneal carcinomatosis of various origins [1]. As more and more sound scientifi c data in this direction are produced and published, peritoneal disease treatment centres have been established in almost every country around the world. Peritoneal surface oncology is a reality. Not every patient with peritoneal carcinomatosis or a primary peritoneal neoplasm is a candidate for a radical combined treatment. Careful patient selection by a dedicated multidisciplinary team is the fi rst requirement for success [2]. The whole justifi cation for a radical locoregional treatment lies in the presumed absence of tumour dissemination beyond the peritoneal cavity, a fact that is frequently observed at a given time point in the natural history of gastrointestinal or gynaecological cancers. And that is the moment at which patients may be considered for a curative-intent treatment. Pseudomyxoma peritonei (PMP) is a fascinating disease characterised by mucinous peritoneal implants and/ or mucinous ascites, originating in the vast majority of cases from a ruptured mucinous appendiceal neoplasm. It is a rare disease, with a reported incidence of one case per million per year. Therefore, it is unlikely that colleagues working in population-based healthcare centres will gain any but anecdotal experience on its diagnosis and treatment. Subsequently, an educational effort to avoid feeding misconceptions on the disease and errors in its appropriate