Abstract

BackgroundPseudomyxoma peritonei (PMP) is a low-grade malignancy characterized by mucinous tumor on the peritoneal surface. Treatment involves cytoreductive surgery (CRS) to remove all macroscopic tumor and perioperative intraperitoneal chemotherapy (PIC) to eliminate remaining microscopic disease. Patients and methodsBetween 1994 and 2009, 93 patients were treated at the Norwegian Radium Hospital with complete CRS and PIC. PIC was administered as early postoperative intraperitoneal chemotherapy (EPIC) using mitomycin C (MMC) and 5-fluoruracil (n = 48) and as hyperthermic intraperitoneal chemotherapy (HIPEC) using MMC (n = 45). Patients were classified into three histopathological subgroups: Disseminated peritoneal adenomucinosis (n = 57), peritoneal mucinous carcinomatosis (n = 21) and an intermediate group (n = 15). Tumor distribution by peritoneal cancer index (PCI) was PCI ≤10 (n = 31), PCI 11–20 (n = 29), PCI ≥21 (n = 33). ResultsRecurrence was diagnosed in 38 patients and 25 patients died during follow-up. Estimated 10-year overall survival (OS) was 69% and 10-year disease-free survival (DFS) was 47%. Mean OS was 154 months (95% CI 131–171) and median OS was not reached (follow-up median 85 months (3–207)). Low-grade malignant histology (p = 0.001) and female gender (p = 0.045) were associated with improved OS. Almost equal OS and DFS were observed between patients treated with EPIC and HIPEC. ConclusionsPatients treated for PMP with complete CRS and PIC achieved satisfactory long-term outcome. The most important prognostic factor was histopathological differentiation, but acceptable survival was observed even in patients with aggressive histology and extensive intraperitoneal tumor growth. Administration of EPIC and HIPEC was equally efficacious with respect to long-term outcome.

Highlights

  • Pseudomyxoma peritonei (PMP) is a rare, low-grade malignancy that usually originates from a ruptured mucinous neoplasm of the appendix

  • In a French multicenter study of patients with PMP treated with cytoreductive surgery (CRS)-hyperthermic intraperitoneal chemotherapy (HIPEC), the number of procedures in the particular hospital was an independent factor for improved outcome, further underlining the importance of high quantity to achieve good quality [264]

  • An important conclusion from these observations combined is that the results of the multimodal treatment of CRS-perioperative intraperitoneal chemotherapy (PIC) seems not to depend on one "expert" surgeon, but rather that organization of the treatment program is the key factor for the outcome in patients with PSM treated with CRS-HIPEC

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Summary

Introduction

Pseudomyxoma peritonei (PMP) is a rare, low-grade malignancy that usually originates from a ruptured mucinous neoplasm of the appendix. An increasing number of patients suffering from peritoneal surface malignancies have been treated with curative intent by a combination of cytoreductive surgery conducted to remove macroscopic tumor and hyperthermic intraperitoneal chemotherapy (HIPEC) to eliminate residual disease. During administration of HIPEC, the peritoneal cavity is connected to a closed circuit that is continuously perfused with a heated chemotherapeutic agent and a carrier solution, thereby providing intraperitoneal residual tumor exposure of the drug at high concentration combined with low systemic absorption and toxicity which is the main dose‐limiting factor. PMP is treated by a two step strategy; surgical cytoreduction aimed to remove all macroscopic tumor and subsequent administration of MMC by hyperthermic intraperitoneal chemotherapy (HIPEC) for elimination of residual microscopic disease and free tumor cells [8,9,10]. Patients with peritoneal surface malignancies are treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy, commonly using mitomycin C (MMC). Oncol. 2014;109:521–526. ß 2013 Wiley Periodicals, Inc

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