Therapy of pseudomyxoma peritonei of appendiceal origin - surgical resection and intraperitoneal chemotherapy

Pseudomyxoma peritonei (PMP) is rare clinical entity presenting with mucus- producing lesions on the peritoneal surface. PMP is found in middle-aged or older patients in approximately two out of 10,000 laparotomies. Most of th e reported cases are due to a primary process located in either appendix or the ovaries, PMP presents with an insidious onset of symptoms. and is char acterized by long-term survival with good general health and absence of vis ceral invasion or distant metastasis. The treatment of choice for PMP is su rgical resection and the removal of free mucus, Despite a high rate of dise ase recurrence, adjuvant or palliative chemotherapy has not vet been clearl y established, In our opinion, intraperitoneal application may be more effe ctive than a systemic regimen because only local treatment can provide suff icient concentrations of chemotherapeutic agents. In view of the lack of st andardized treatment regimens and based on our experience, we recommend int raperitoneal chemotherapy with 5-FU and mitomycin C or cisplatin. We report ten cases of PMP of appendiceal origin and present a review of th e literature on this disease and its treatment.