The therapeutic approach combining cytoreductive surgery with perioperative intraperitoneal chemotherapy achieved long-term survival in a selected group of patients with PC from colorectal origin with acceptable morbidity and mortality. The complete cytoreductive surgery was the most important prognostic indicator.

The combined modality strategy for PMP may be performed safely with acceptable morbidity and mortality in a specialized unit setting with 63% of patients surviving beyond 10 years. Minimizing nondefinitive operative and systemic chemotherapy treatments before definitive cytoreduction may facilitate the feasibility and improve the outcome of this therapy to achieve long-term survival. Optimal cytoreduction achieves the best outcomes.

The data suggest that CRS combined with HIPEC achieved prolonged survival in selected patients with DMPM.

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532 Background: Pseudomyxoma peritonei (PMP) originating from an appendiceal mucinous neoplasm remains a biologically heterogeneous disease. The purpose of this study was to evaluate the outcome and long-term survival after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) consolidated through an International Registry study. Methods: A retrospective multi-institutional registry was established through collaborative efforts of participating units affiliated with the Peritoneal Surface Oncology Group International (PSOGI). Results: 2298 patients from 16 specialized units underwent CRS for PMP. Treatment related mortality was 2% and major operative complication was 24%. The median survival was 196 months (16.3 years) and the median progression-free survival was 98 months (8.2 years) with a 10- and 15-year survival rate of 63% and 59% respectively. Multivariate analysis identified prior chemotherapy treatment (P<0.001), PMCA histopathological subtype (P<0.001), major postoperative complication (P=0.008), high PCI (P=0.013), debulking surgery (CCR2/3) (P<0.001), not using HIPEC (P=0.030) as independent predictors for a poorer progression-free survival. Older age (P=0.006), major postoperative complication (P<0.001), debulking surgery (CCR2/3) (P<0.001), prior chemotherapy treatment (P=0.001) and PMCA histopathological subtype (P<0.001) were independent predictors of a poorer overall survival. Conclusions: The combined modality strategy for PMP may be performed safely with acceptable morbidity and mortality in a specialized unit setting with 63% of patients surviving beyond 10-years. Minimizing non-definitive operative and systemic chemotherapy treatments prior to definitive cytoreduction may facilitate the feasibility and outcome of this therapy to achieve long-term survival. Optimal cytoreduction achieves the best outcomes.

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.

Favorable outcome after comprehensive treatment can be expected in patients with DPAM, not treated with preoperative systemic chemotherapy and amenable to adequate cytoreduction. MUC-2, CK-20, and CD44s expression may be related to PMP unique biologic behavior.