Pathophysiology and classification of pseudomyxoma peritonei

Background: The term pseudomyxoma peritonei (PMP) was first described in 1884 and there has been much debate since then over the term. A recent consensus of world experts agreed that PMP should be thought of as a clinical entity characterised by the presence of mucinous ascites, omental cake, peritoneal implants and possibly ovarian involvement. It generally originates from mucinous appendiceal tumours. Content: This review details the clinical presentation of this unusual condition, presents the new classification system and how this relates to outcome. The pathophysiology of this disease is also explored with a special reference to the relationship of the disease to tumour markers. Summary: A classification system has been agreed upon by the leading experts in PMP which is now divided into low and high grade mucinous carcinomatosis peritonei. This distinction correlates with clinical outcome as does the presence of raised tumour markers preoperatively. Outlook: Research needs to be focused on understanding the factors associated with poor prognosis through well designed multi-centred prospective studies. This will allow us to identify patients with bad tumour biology so that targeted treatment based on likely prognosis may then become a reality.