Prognostic Factors in Pseudomyxoma Peritonei with Emphasis on the Predictive Role of Peritoneal Cancer Index and Tumor Markers

SIMPLE SUMMARY: Disease outcome of patients with pseudomyxoma peritonei (PMP) and appendiceal neoplasms improved gradually in the past decades, as their pathology is better understood, together with a refined subtypisation. The introduction of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in the treatment of well-selected patients has led to significantly better outcomes compared to “classical” therapies alone. Due to the fact that PMP is considered a rare malignancy, real-word cross-sectional studies are highly warranted to establish reliable prognostic and predictive factors in large and uniform patient populations. Apart from established prognostic indicators, such as the peritoneal cancer index, the extent of cytoreduction and the plasma level of tumor markers are as well of significance. We aimed to refine and confirm the validity of these factors in our whole patient population and in patients with low-grade histologies. Our study can refine and improve our current understanding of PMP management on a sizeable cohort. ABSTRACT: Background: Pseudomyxoma peritonei (PMP) is a rare peritoneal condition where mucus-secreting tumorous cells progressively produce a thick, gelatin-like substance. The prognosis of patients with PMP is determined by the degree of cellularity within the mucin (low-grade (LAMN) vs. high-grade (HAMN) histologic features) and by the extent of the disease. Methods: Prognostic relevance of tumor markers CA19-9 and CEA, gender, Peritoneal Cancer Index (PCI), and completeness of cytoreduction (CC) after cytoreductive surgery were evaluated on 193 consecutive PMP patients, based on a retrospective analysis of prospectively gathered data from a German tertial referral center. Results: We demonstrated that low PCI, CC0 status, low-grade histology, and female gender were independent positive prognostic factors for both overall survival (OS) and progression-free survival (PFS). Furthermore, LAMN patients with achieved CC0 status show significantly better OS and PFS compared to those with CC1 status (p = 0.0353 and p = 0.0026 respectively). In contrast, the duration and drug of hyperthermic intraperitoneal chemotherapy (HIPEC) were not prognostic in any comparison. Increased CA19-9 and CEA levels were significantly associated with HAMN cases, but also predicted recurrence in patients with low-grade histologies. Conclusion: Our study confirmed the prognostic role of tumor markers and emphasized the importance of CC status and PCI in a large cohort of PMP- and LAMN patients.