Pseudomyxoma peritonei (gelatinous peritonitis )]

Pseudomyxoma peritonei (P.P.) is characterised by the presence in the peritoneal cavity of 3 elements--mucinous neoplasic cells, mucinous ascites and diffuses mucinous implants. We analysed the patients treated in our service and we made a literature review. The study is a retrospective analysis on the 1984-2000 period. 6 patients were diagnosed with P.P., 2 females and 4 men, with medial age 63 years (53-75). Clinical findings and biologic analysis were not specific and the imagistic explorers didn't permit the preoperative diagnosis. The patients were submitted to variate surgical procedures, based on the benign (4 cases) or malignant (2 cases) character of the disease and on the origin of the lesions: cystadenoma of the appendix with secondary tumours of the ovary (the 2 females) and, respectively, cystadenoma and cytsadenocarcinoma of the appendix, mucinous paraenteric cyst with pseudomyxoma retroperitonei, mucinous recto-sigmoidian neoplasm (the 4 men). We practiced intraperitoneal chemotherapy with Thio-Tepa in 5 cases (intraoperative in 4 cases) and systemic, with 5-FU and mytomicine, in one case. The operative morbidity and mortality were null. All the patients were follow-up (average 4.2 years). The global 5-year survival rate was 60% (100% in the benign disease and 0% in the malignant form).P.P. is a rare disease, still poorly understood. The origin of the disease is the appendix (70-80%) and less frequently the ovary. It was divided in two distinct forms: disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. The treatment associates radical surgery and intraperitoneal chemohyperthermia, in specialised centres, but the prognosis still remains poor (50-70% 5-year global survival rate).