Consensus statement on the loco‐regional treatment of appendiceal mucinous neoplasms with peritoneal dissemination (pseudomyxoma peritonei)

Abstract

Pseudomyxoma peritonei (PMP) is a rare condition mostly originating from low malignant potential mucinous tumours of the appendix. Although this disease process is minimally invasive and rarely causes haematogenous or lymphatic metastases, expectation of long-term survival are limited with no prospect of cure. Recently, the combined approach of cytoreductive surgery (CRS) and perioperative loco-regional chemotherapy (PLC) has been proposed as the standard of treatment for the disease. The present paper summarizes the available literature data and the main features of the comprehensive loco-regional treatment of PMP. The controversial issues concerning the indications and technical methodology in PMP management were discussed through a web-based voting system by internationally known experts. Results were presented for further evaluation during a dedicated session of "The Fifth International Workshop on Peritoneal Surface Malignancy (Milan, Italy, December 4-6, 2006)". The experts agreed that multiple prospective trials support a benefit of the procedure in terms of improved survival, as compared with historical controls. Concerning the main controversial methodological questions, there was an high grade of consistency among the experts and agreement with the findings of the literature.

Mucinous epithelial neoplasms of the appendix and pseudomyxoma peritonei

“…One of the most important factors is attaining complete cytoreduction, which is achieved in 40-91% of cases. 87,88 In one study, patients who had complete cytoreduction had a 5-and a 10-year survival rate of 87% and 74%, respectively, whereas those in which complete cytoreduction could not be achieved had 34% and 23% 5-and 10-year survival rates. 87 In this regard, the experience of the surgeon may have a role, with specialized centers achieving better survival statistics.…”

Cytoreductive surgery (SRC) for treatment of peritoneal carcinomatosis and hyperthermic ıntraperitoneal chemotherapy (HIPEC): Our initial experience and technical details

“…In a multicenter study on 2296 patients treated with CRS and HIPEC, the median survival has been reported as 196 months, and 10-year overall survival rate as 63% (63). In the same study, the mean (64). Pseudomyxoma peritonei is the disease with the best response to this treatment regardless of the PCI score.…”

Abdominal Advanced Oncologic Surgery

“…Results are encouraging with a 5-ys-OS ranging from 62.5 to 100% for low grade PMP and from 0 to 65% for high grade disease . This differentiation is crucial for the evolution of disease; low grade tumours are slow growing and indolent differently from those high grade, fast growing and aggressive (Sugarbaker and Chang, 1999;Witkamp et al, 2001;Güner et al, 2005;Moran et al, 2008;Moran and Cecil, 2003;Murphy et al, 2007;Baratti et al, 2008;Elias et al, 2008;Loungnarath et al, 2005;Smeenk et al, 2007;Stewart et al, 2005;Yan et al, 2006). The goal of the surgical cytoreduction is to remove all the visible tumor by the following procedures: right subdiaphragmatic and parietal peritonectomy, left subdiaphragmatic and parietal peritonectomy,greater omentectomy with splenectomy, lesser omentectomy and stripping of the omental bursa, andpelvic peritonectomy with salpingo-oophorectomy in women.…”