Abstract： Objective To investigate the management of pseudomyxoma peritonei originated in the appendix.Methods The clinical data of 51 patients with pseudomyxoma peritonei originated in the appendix who were admitted to the Beijing Hospital from 1970 to 2010 were retrospectively analyzed.The results of operation,reoperation,adjuvant treatment and follow-up were analyzed.The time from pseudomyxoma peritonei recurrence to the reoperation between patients who did or did not receive chemotherapy was compared by two tailed t test.Results Of the 51 patients,48 received operation,and the operation time was (135 ± 72 )minutes.Tumor recurrence was observed in 34 patients,and 16 of them received cytoreduction procedure,and 33 cytoreduction procedures were performed in total.The median time of follow-up was 49.7 months (range,3-132 months).The disease-specific survival was observed in 25 patients and disease-free survival in 16 patients.Four patients died of tumor recurrence or progression.The results of postoperative pathological examination confirmed that 19 patients were with benign disseminated peritoneal adenomucinosis (DPAM),26 were with malignant peritoneal mucinous carcinomatosis (PMCA) and 6 were with intermediate subtype (PMCA-1).The 3-,5- and 10-year survival rates were 75％ (38/51),55％ (28/51) and 22％ ( 11/51 ),respectively.The survival time and reoperation time interval for patients who received postoperative chemotherapy were ( 21 ± 4) months and ( 10 ± 6 ) months,which were longer than (19 ±7 )months and (7 ±4)months of those who did not receive postoperative chemotherapy (t =1.027,0.361,P ＞ 0.05).The median survival time of patients with benign DPAM,PMCA-1 and malignant PMCA were 96,63,23 months,respectively.The tumor recurrence interval for patients with benign DPAM and those with malignant PMCA were ( 15 ± 5 ) months and (7 ± 4) months,with significant difference ( t =2.193,P ＜ 0.05 ).Conclusions An active cytoreduction surgery is feasible for patients with pseudomyxoma peritonei originated in the appendix in improving survival.Repeated cytoreduction is a treatment of strategy to prolong the recurrence time and improve the prognosis of selected patients.