Early Data Of Patients With Pseudomyxoma Peritonei From Appendiceal Origin Treated By A Strategy Of Cytoreductive Surgery And Hyperthermic Intraperitoneal Chemotherapy

Background. Appendiceal peritoneal pseudomyxoma (PMP) is very rare disease and its longterm prognosis is poor. The aim of this study was to evaluate the results of an aggressive treatment approach used in our institution for the last 4 years. Methods. We selected all patients with PMP from appendiceal origin who were treated by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) at the Klaipeda University Hospital between January 2012 and January 2016. Data from all patients with PMP arising from the appendix were retrospectively collected and analyzed. Treatment consisted of complete surgical cytoreduction , followed by hyperthermic intraperitoneal chemotherapy with mitomycin at 42°C over 90 minutes. Ronnett’s hystologic classification was used for tumor grading. Results. A total of 6 patients underwent cytoreduction and peritonectomy plus HIPEC. Median age at diagnosis was 57 years (range, 39-67). All our patients were female. The previous surgery score at the moment of admission was PSS-2 for three patients, PSS-3 for 3 patients. Four patients were diagnosed as diffuse peritoneal adenomucinosis (DRAM) and two as peritoneal mucinous carcinomatosis( PMCA). In all of the patients, optimal cytoreduction CC-0 (5 patients) and CC-1 (one patient) was achieved. The median peritoneal cancer index (PCI) was 17 (range, 14-25) as an indicator of disease extension.The median number of visceral resections performed per patient was 3 (range, 1-5). The median duration of CRS/HIPEC was 8 hours and 10 minutes (range, 7 hours and10 min. to 9 hours 20 min). Mean postoperative stay was 13 days (range, 8-18). The 30 days postoperative and in-hospital mortality were zero . One patient experienced temporary haemorhagic cystitis. The mean follow-up period was 28 months ( range, 8-45). At the time of analysis all patients are alive and without recurrence. Conclusions PMP from appendiceal origin can be treated with curative intent in a large percentage of cases by cytoreductive surgery associated with HIPEC. This new approach could be performed safely with acceptable morbidity and mortality in selected patients treated in specialized centers.