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Page 2 of 12     Yonemura et al. J Cancer Metastasis Treat 2022;8:43  https://dx.doi.org/10.20517/2394-4722.2022.49

               Keywords: Peritoneal mesothelioma, mesothelioma, intraperitoneal chemotherapy, HIPEC, peritonectomy




               INTRODUCTION
               Peritoneal mesothelioma (PM) is a rare peritoneal malignancy characterized by diffuse involvement of the
               peritoneal surface. It develops from mesothelial cells. Asbestos, talc, SV 40 virus, and chronic peritonitis are
               implicated as factors in the development of PM .
                                                       [1-3]
                                                                                         [4-7]
               About 33%-50% of patients diagnosed with PM report known prior exposure to asbestos .

               PM shows a lower male-to-female ratio and a lower mean age at death in comparison with pleural
                           [5,6]
               mesothelioma . Age-standardized incidence rates generally range from 0.5 to 3 cases per million
               population  with a male-to-female ratio of 1.6:1 . The highest rates are reported in the UK, Australia, and
                                                        [5]
                        [6]
                          ,
                                                   [7-9]
               New Zealand, with the lowest rate in Japan . Now that asbestos usage has been banned by law, the number
                                              [8]
               of PM deaths is expected to decrease .
               Conventional classification comprises diffuse malignant peritoneal mesothelioma (DMPM), multicystic
                                                                                                        [9]
               peritoneal mesothelioma (MCPM), and well-differentiate papillary peritoneal mesothelioma (WDPPM) .
               MCPM has a low malignant potential with a favorable prognosis, and WDPPM shows less aggressive
               potential than DMPM, because it tends not to invade subperitoneal tissue and has weak proliferative
               activity .
                     [10]

               In the past, DMPM has been regarded as a rapidly lethal disease because of its high capacity for invasion
               and lymph node metastasis. In addition, systemic chemotherapy (SC) has no effect on the long-term
               survival of patients with DMPM.

               Recently, surgical treatment combined with hyperthermic intraperitoneal chemoperfusion (HIPEC) has
               emerged as a promising treatment that might improve survival and cure rates .
                                                                                [11]

               The purpose of this manuscript is to review both the efficacy of locoregional treatment for patients with
               DMPM and WDPPM using cytoreductive surgery (CRS) and HIPEC and the criteria for treatment selection
               by reviewing articles on more than 50 DMPM patients.


               TREATMENT MODALITIES FOR PM
               Several treatments have been reported, including SC, CRS, the combination of CRS plus perioperative
               chemotherapy  (POC)  including  neoadjuvant  systemic  chemotherapy  (NASC),  neoadjuvant
               intraperitoneal/systemic chemotherapy (NIPS), laparoscopic neoadjuvant HIPEC, intraoperative HIPEC,
               early postoperative intraperitoneal chemotherapy (EPIC), and non-hyperthermic intraoperative
               chemotherapy (NIPEC) [10,12] . Among these treatments, CRS combined with POC is nominated as the most
               effective treatment to improve survival in DMPM patients with a low preoperative peritoneal cancer index
               (PCI) ≤ 19 . However, there have been no randomized phase III trials evaluating surgical treatment
                         [10]
               combined with POC in the past.


               When the PCI is less than the threshold cutoff level determined by laparoscopy, positron emission
               tomography (PET), and/or computed tomography (CT), CRS should be performed after NASC or NIPS.
               However, chemotherapy is recommended in DMPM patients with PCI above cutoff levels or diffuse
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