Operative findings, early complications, and long-term survival in 456 patients with pseudomyxoma peritonei syndrome of appendiceal origin.

摘要: BACKGROUND: Pseudomyxoma peritonei syndrome is a clinical entity characterized by mucinous ascites usually originating from perforated appendiceal tumor. Currently optimal therapy considered complete macroscopic tumor removal (complete cytoreduction) combined with heated intraperitoneal chemotherapy. When cytoreduction not achievable, major debulking undertaken. The long-term follow-up of patients undergoing surgery for tumors in national pseudomyxoma center reported. METHODS: Between March 1994 and July 2009, 456 underwent surgery. treatment strategy involved combination cytoreductve aiming removal, hyperthermic chemotherapy mitomycin C. Where was possible, maximal performed. Perioperative outcomes predicted 5- 10-year survival are RESULTS: Overall 15 (3%) were resectable at laparotomy. Of the 441 who had resection, 289 (66%) 152 (34%) debulking. Postoperative in-hospital mortality 1.6% grade 3/4 morbidity 7%. Kaplan-Meier method overall 69% 57%, respectively. Five-and 87% 74% compared 34% 23% CONCLUSION: Complete achieved peritoneal dissemination good probable cure more than two-thirds.