[Pseudomyxoma peritonei--a syndrome with varying prognosis]

Pseudomyxoma peritonei (PMP) is an unusual condition in which massive amounts of gelatinous fluid collections are associated with mucinous implants on the peritoneal and omental surfaces. Recent molecular genetic studies suggest the appendix as the most common primary origin of this disease. Surgical debulking in a combination with intraperitoneal (i.p.) chemotherapy has been advocated to treat this disease. Thirty patients were treated for PMP, by this concept, at Uppsala University hospital between 1993 and April 2003. One patient died because of septic complications (inhospital mortality 3 per cent). Twenty-nine patients are alive after median follow-up of 16 months (range 1-92), 18 of them tumor free.