title: Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei and Appendix Tumours.
abstract: Pseudomyxoma peritonei (PMP) is the intra-peritoneal accumulation of mucus due to mucinous neoplasia, most often from a ruptured mucinous appendiceal neoplasm. A similar syndrome is caused by appendix cancer and other gastrointestinal malignancies. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) provides long-term survival in selected patients with these conditions. The management of the appendiceal neoplasm prior to development of peritoneal involvement is initially discussed. This is followed by an overview of the management of peritoneal disease caused by appendiceal neoplasms. The principles and basic techniques of CRS and intraperitoneal chemotherapy (both intraoperative and post operative) are then discussed. Survival outcomes from several large studies are summarised. Prognostic factors are also discussed. We report our basic outcome data for the 345 patients with PMP or appendix cancer treated at our institution. Finally, the promising upcoming treatment of mucolytic therapy is discussed. We conclude that appendiceal neoplasms, although rare can cause significant morbidity and mortality. With optimal management long-term survival is possible in the majority of patients. The key to treatment is complete cytoreduction and use of hyperthermic intraperitoneal chemotherapy.
authors.affiliation: Department of Surgery, University of New South Wales, Sydney, New South Wales ; Department of Surgical Oncology, St George Hospital, Sydney, NSW Australia.
authors.forename: Joshua
authors.initials: J
authors.lastname: Lansom
authors.affiliation: Department of Surgery, University of New South Wales, Sydney, New South Wales ; Department of Surgical Oncology, St George Hospital, Sydney, NSW Australia.
authors.forename: Nayef
authors.initials: N
authors.lastname: Alzahrani
authors.affiliation: Department of Surgical Oncology, St George Hospital, Sydney, NSW Australia ; Cancer Care Centre, St George Hospital, Sydney, NSW Australia.
authors.forename: Winston
authors.initials: W
authors.lastname: Liauw
authors.affiliation: Department of Surgery, University of New South Wales, Sydney, New South Wales ; Department of Surgical Oncology, St George Hospital, Sydney, NSW Australia.
authors.forename: David L
authors.initials: DL
authors.lastname: Morris
keywords: Appendiceal neoplasms; Cytoreductive surgery; HIPEC; Hyperthermic intraperitoneal chemotherapy; Mucocoele; PMP; Peritoneal surface malignancy; Peritonectomy; Pseudomyxoma peritonei; Surgery; Surgical oncology