Surgical and multimodal therapy of Pseudomyxoma peritonei

The rarity of Pseudomyxoma peritonei, its complex biology and the remaining inconsistencies in terminology all contribute to rendering therapeutic rec ommendations a difficult task. In principle, a multimodal concept combining cytoreductive surgery with peri- and postoperative intraperitoneal chemoth erapy constitutes the preferable treatment. Initially, surgical peritonecto my aims to achieve the complete removal of tumor cells at the macroscopic l evel. Subsequently in the early postoperative phase when the absence of adh esions allows for a homogeneous intraabdominal spread of cytotoxic drugs, i ntraperitoneal application of 5-fluorouracil and mitomycin C represents the most solidly established treatment. Therapeutic failures are often due to insufficiently radical cytoreductive surgery or a lack of homogeneous distr ibution of cytotoxic drugs in the abdomen. In view of the rarity of their c ondition, patients with Pseudomyxoma peritonei should be treated at or in c onjunction with specialized centers. Only such a "center-oriented" approach will secure the standardization of treatment and help to clarify unsettled therapeutic questions. The development of improved therapeutic concepts wi ll depend on concise histopathological classification. Already at present t herapeutic decisions should not only be be based on the clinical aspect of a "mucinous abdomen" but should be guided by the pathological differentiati on between disseminated peritoneal adenomucinosis (DPAM), with a relatively good prognosis, and the more aggressive peritoneal mucinous carcinomatosis (PMCA).