In the large majority of cases, the cause is a ruptured appendiceal mucinous adenoma and the gelatinous matrix is acellular. Whether the ovary is a second primary source of the disease or is secondarily involved is controversial. A smaller percent of patients have a mucinous cystadenocarcinoma as the cause, in which case malignant cells are found in the mucous. The tumor usually arises in the appendix, rarely in other organs, is indolent and rarely metastasizes. The development of pseudomyxoma is slow and insidious, and patients often present with few symptoms other than abdominal distention.
Progression often leads to intestinal obstruction.
The prognosis is more dependent on the degree of intraperitoneal dissemination than on histology. The mode of death is usually intestinal obstruction and malnutrition. All rights reserved.