PSEUDOMYXOMA PERITONEI

Pseudomyxoma peritonei results from implantation of malignant tumors o r irritation from ruptured benign cysts. This disease is traditionally characterized by accumulation of mucinous ascites, relatively long su rvival period and absence of extraperitoneal metastases. Disease progr ession is difficult to predict because of the spectrum of underlying p athologic entities. Four unusual instances of pseudomyxoma peritonei a re presented. An instance of the neoplasm confined to die splenic pare nchyma suggests potential for hematogenous dissemination. The tumor ca n be limited to and extend along the retroperitoneum. Retained rectal tissue after proctocolectomy may be a possible origin of disease. Ente robronchial fistula formation is a serious long term complication. Agg ressive surgical approach with resection of the bulk of disease offers the optimal palliation and prognosis.