Abstract

BackgroundComplete cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is the only curative treatment for pseudomyxoma peritonei (PMP) arising from the appendix. High peritoneal carcinomatosis index (PCI) is associated with an increased risk of surgical complications. The objective of this study was to present the results of a planned two-step surgical strategy to decrease postoperative morbidity and improve resectability of patients with very high PCI.MethodsAll consecutive patients who underwent a planned two-step surgical approach for PMP between January 2012 and March 2020 were retrospectively included. This approach was offered for patients with low-grade PMP with PCI > 28 for which feasibility of a complete CRS in one operation was uncertain. The first surgery included a complete CRS of the inframesocolic compartment and omentectomy. HIPEC was delivered at the second surgery, after complete CRS of the supramesocolic compartment. Postoperative morbidity was assessed using the Clavien-Dindo classification and survival results were also collected.ResultsEight patients underwent the two-step approach. The median PCI was 33 (29–39) and the median time between the two procedures was 111 days (90–212 days). One patient was deemed unresectable at the second surgery. The rate of major morbidity was 0% for the first step and 25% for the second step, with no mortality. Median follow-up was 53.8 months (3–73 months).ConclusionA two-step surgical management for low-grade PMP patients with very high PCI is safe and feasible, with acceptable postoperative morbidity and no compromise on oncological outcomes.

Highlights

  • Pseudomyxoma peritonei (PMP) is a rare condition characterized by intraperitoneal dissemination of mucinous neoplasia affecting about 1 patient per million population per year [1]

  • This approach was considered only for low-grade pseudomyxoma peritonei (PMP) patients (DPAM, peritoneal mucinous carcinomatosis (PMCA)-I, or low-grade appendiceal mucinous neoplasms (LAMN)) with very extensive disease, which can be defined as a peritoneal carcinomatosis index (PCI) ≥ 28 [17], in which complete cytoreductive surgery (CRS) was deemed achievable with two surgeries and for which the disease was presumed to be indolent and unlikely to flare up between procedures

  • This study reports the outcome of 8 PMP patients with very high PCI who were approached with a two-step surgical strategy in an attempt to reduce the postoperative morbidity and mortality associated with CRS for such an extensive disease

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Summary

Introduction

Pseudomyxoma peritonei (PMP) is a rare condition characterized by intraperitoneal dissemination of mucinous neoplasia affecting about 1 patient per million population per year [1]. Its main origin is the appendix, but it may arise from the ovaries or other intraperitoneal organs. This disease is usually slowly progressive, its natural course is fatal due to increased abdominal distension, intestinal obstruction by extrinsic compression, or respiratory failure [2].

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