Pseudomyxoma peritonei.

Pseudomyxoma peritonei results from implantation of malignant tumors or irritation from ruptured benign cysts. This disease is traditionally characterized by accumulation of mucinous ascites, relatively long survival period and absence of extraperitoneal metastases. Disease progression is difficult to predict because of the spectrum of underlying pathologic entities. Four unusual instances of pseudomyxoma peritonei are presented. An instance of the neoplasm confined to the splenic parenchyma suggests potential for hematogenous dissemination. The tumor can be limited to and extend along the retroperitoneum. Retained rectal tissue after proctocolectomy may be a possible origin of disease. Enterobronchial fistula formation is a serious long term complication. Aggressive surgical approach with resection of the bulk of disease offers the optimal palliation and prognosis.