Survey on the incidence and management of pseudomyxoma peritonei in Japan.

ABSTRACT

PURPOSE: Pseudomyxoma peritonei(PMP)is a rare condition characterized by massive ascites accumulation due to mucinous tumor dissemination in the peritoneal cavity. More recently, Sugarbaker has defined radical cytoreductive surgery and intraperitoneal chemotherapy as standardized therapy for PMP. The objective of this study was to investigate the incidence of PMP and the recent therapeutic approaches for this condition in Japan. METHODS: Questionnaires answered by PMP patients between 2006 and 2010 were evaluated in this study. The study included 1, 084 gastroenterological surgery and gynecology institutions in Japan. RESULTS: Data from 379 institutions were analyzed(response rate 35. 0%). The mean number of diffuse PMP cases at a single institution in 5 years was 0. 78. Of 266 diffuse cases, surgery was performed in 232 cases(87. 2%)and chemotherapy was administered in 138 cases(51. 9%). However, complete cytoreduction was achieved in only 31 of 232 operated cases(13. 4%)and intraperitoneal chemotherapy was administered to only 45 of 138 patients receiving chemotherapy( 32. 6%). CONCLUSION: Despite the limited data, our results suggest that PMP occurs rarely in Japan, similar to the trend reported in Western countries. Further, the Sugarbaker procedure was not widely used in Japanese institutions.