Abstract
Pseudomyxoma peritonei is a rare disease characterized by a large amount of mucinous ascites with peritoneal and omental implants. The etiology of the disease remains unclear. Different histological categories have been described: the benign, malignant, and the intermediate forms. It is commonly diagnosed incidentally at laparotomy. Most investigators agree that radical surgical debulking and appendectomy is the cornerstone of treatment, but the optimal management of the disease remains controversial. The role of intraoperative and intraperitoneal chemotherapy has been evaluated by a number of authors. The clinical outcomes vary widely between the different histological types and treatment modalities.
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