Peritoneal pseudomyxoma: results of a systematic policy of complete cytoreductive surgery and hyperthermic intraperitoneal chemotherapy

Abstract

Abstract Background Pseudomyxoma peritonei (PMP) is characterized by progressive intraperitoneal accumulation of mucous and mucinous implants, usually derived from a ruptured, possibly malignant mucinous neoplasm of the appendix. Treatment based on complete cytoreductive surgery (CCRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) is gaining support. The aim of this study was to identify pre- and perioperative factors of prognostic value. Methods A total of 105 patients (with no residual tumours exceeding 2 mm) were treated with CCRS plus HIPEC based on oxaliplatin. Clinical, radiological, pathological factors and blood markers were analysed to determine their prognostic value for survival. Results Mortality (7·6 per cent) and morbidity (67·6 per cent) were significantly correlated with peritoneal index, pathological grade and blood CA19·9 level. The median follow-up was 48 months. Seven patients died after hospital discharge. Overall and disease-free 5-year survival rates were 80·0 and 68·5 per cent respectively. The Cox model identified only two significant factors impacting on disease-free survival: CA19·9 level and pathological grade. Conclusion CCRS is the most effective treatment for PMP, and adding HIPEC prolongs long-term survival. Further strategies should focus on improving postoperative outcome in extended PMP.