Multisocietal Consensus on the use of Cytoreductive Surgery and HIPEC for the Treatment of Pseudomyxoma Peritonei: A GRADE Approach for Evidence Evaluation and Recommendation.

Abstract

Introduction: Pseudomyxoma peritonei (PMP) is a rare malignancy characterized by mucinous ascites and peritoneal implants, primarily arising from ruptured appendiceal neoplasms. Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has become the preferred treatment for resectable and operable cases.

Methods: An international panel from the Peritoneal Surface Oncology Group International (PSOGI), the International Society for the Study of Pleura and Peritoneum (ISSPP), the Society of Surgical Oncology (SSO), the European Society of Surgical Oncology (ESSO), and EURACAN used the GRADE methodology and ADOLOPMENT approach to adapt existing guidelines. Clinical questions were framed using the PICO framework, and systematic reviews were conducted. Evidence certainty and the balance of benefits and harms were evaluated with the Evidence-to-Decision framework.

Results: A strong recommendation was made for CRS and HIPEC over debulking surgery for resectable PMP. Observational studies reported 5- and 10-year survival rates of 80% and 55%, respectively, with CRS and HIPEC, compared to lower outcomes with debulking. Despite the very low certainty of evidence, the panel strongly endorses CRS and HIPEC over debulking surgery. This recommendation is driven by PMP's known chemoresistance, the lack of viable alternative treatments, and the survival benefits observed in observational studies coming only from radical surgery.

Conclusion: This consensus endorses CRS and HIPEC as the standard of care for operable and resectable PMP. This recommendation will increase acceptance and awareness among a broader audience of healthcare professionals about the potential benefits of this treatment in managing this rare peritoneal disease.