Pseudomyxoma peritonei in a hernia sac: analysis of 20 patients in whom mucoid fluid was found during a hernia repair

Introduction: Pseudomyxoma peritonei syndrome is a rare disease that causes extensive accumulation of noninvasive mucinous tumour and mucinous fluid t hroughout the abdomen and pelvis, The primary tumour is a perforated append iceal adenoma or mucinous adenocarcinoma. In patients with an open processu s vaginalis or other peritoneal defect, the adenomatous epithelial cells ma y accumulate within the hernia sac, causing mucoid fluid to be present at t he 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 m ucoid material was found in a hernia sac during a hernia repair. Clinical f eatures 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 impac t on the outcome, A lack of interim events vs the use of interim treatments between hernia repair and definitive cytoreduction was significant in rela tion to a complete vs incomplete cytoreduction. Non-aggressive histology an d an interval of less than I year was statistically significantly associate d 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 importa nt. When adenomatous epithelial cells are present, a CT scan should be perf ormed to look for the typical distribution of mucinous tumour and fluid tha t suggests the pseudomyxoma peritonei syndrome. If the diagnosis can be con firmed, referral to a centre experienced in the treatment of this rare dise ase is suggested, (C) 2001 Harcourt Publishers Ltd.