Introduction: Pseudomyxoma peritonei syndrome is a rare disease that causes extensive accumulation of non-invasive mucinous tumour and mucinous fluid throughout the abdomen and pelvis. The primary tumour is a perforated appendiceal adenoma or mucinous adenocarcinoma. In patients with an open processus vaginalis or other peritoneal defect, the adenomatous epithelial cells may accumulate within the hernia sac, causing mucoid fluid to be present at the time of hernia repair. Materials and methods: From February 1991 to September 1997, the diagnosis of pseudomyxoma peritonei syndrome was established in 20 patients in whom mucoid material was found in a hernia sac during a hernia repair. Clinical features of these patients were critically evaluated using the completeness of cytoreduction as an endpoint. Results: The treatments delivered at the time of hernia repair had no impact on the outcome. A lack of interim events vs the use of interim treatments between hernia repair and definitive cytoreduction was significant in relation to a complete vs incomplete cytoreduction. Non-aggressive histology and an interval of less than 1 year was statistically significantly associated with a favourable outcome from surgical intervention. Conclusion: When mucoid fluid is encountered at the time of a hernia repair, recovery of that fluid and the hernia sac for histologic study is important. When adenomatous epithelial cells are present, a CT scan should be performed to look for the typical distribution of mucinous tumour and fluid that suggests the pseudomyxoma peritonei syndrome. If the diagnosis can be confirmed, referral to a centre experienced in the treatment of this rare disease is suggested.
- Cytoreductive surgery
- Hernia sac
- Intraperitoneal chemotherapy adenomucinosis
- Pseudomyxoma peritonei syndrome
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