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Appendiceal tumours and pseudomyxoma peritonei: Literature review with PSOGI/EURACAN clinical practice guidelines for diagnosis and treatment

Published:February 27, 2020DOI:https://doi.org/10.1016/j.ejso.2020.02.012

      Abstract

      Pseudomyxoma Peritonei (PMP) is a rare peritoneal malignancy, most commonly originating from a perforated epithelial tumour of the appendix. Given its rarity, randomized controlled trials on treatment strategies are lacking, nor likely to be performed in the foreseeable future. However, many questions regarding the management of appendiceal tumours, especially when accompanied by PMP, remain unanswered. This consensus statement was initiated by members of the Peritoneal Surface Oncology Group International (PSOGI) Executive Committee as part of a global advisory role in the management of uncommon peritoneal malignancies. The manuscript concerns an overview and analysis of the literature on mucinous appendiceal tumours with, or without, PMP. Recommendations are provided based on three Delphi voting rounds with GRADE-based questions amongst a panel of 80 worldwide PMP experts.

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      Glossary

      CA 125
      cancer antigen 125
      CA 19.9
      cancer antigen 19.9
      CC0
      no visible disease after CRS
      CC1
      remaining nodules <2.5 mm after CRS
      CCRS
      complete cytoreductive surgery
      CEA
      carcino-embryonic antigen
      CPM
      colorectal peritoneal metastases
      CRS
      cytoreductive surgery
      DFS
      disease free survival
      EPIC
      Early postoperative intra-peritoneal chemotherapy
      GCC
      goblet cell carcinoid
      HAMN
      high grade appendiceal mucinous neoplasms
      HIPEC
      Hyperthermic Intra Peritoneal Chemotherapy
      LAMN
      low grade appendiceal mucinous neoplasms
      LoE
      Level of Evidence
      MTD
      maximum tumour debulking
      OS
      overall survival
      PFS
      progression free survival
      PMP
      pseudomyxoma peritonei
      RHC
      right hemicolectomy
      SoR
      Strength of Recommendation
      SRC
      signet ring cell