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Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei and Appendix Tumours
Joshua Lansom1, Nayef Alzahrani1, Winston Liauw2
1Department of Surgery, University of New South Wales, Sydney, New South Wales ; Department of Surgical Oncology, St George Hospital, Sydney, NSW Australia.
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.
Insights
Pseudomyxoma peritonei (PMP) management involves cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for long-term survival. Optimal treatment of appendiceal neoplasms, even rare ones, offers significant survival benefits.
Area of Science:
- Oncology
- Surgical Oncology
- Gastroenterology
Background:
- Pseudomyxoma peritonei (PMP) is characterized by intra-peritoneal mucus accumulation, often originating from appendiceal neoplasms.
- Appendix cancer and other gastrointestinal malignancies can also lead to similar peritoneal disease.
- Cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) offers a chance for long-term survival in select patients.
Purpose of the Study:
- To discuss the management of appendiceal neoplasms before and after peritoneal involvement.
- To outline the principles and techniques of CRS and intraperitoneal chemotherapy.
- To present institutional outcome data and discuss future therapeutic options like mucolytic therapy.
Main Methods:
- Review of management strategies for appendiceal neoplasms and peritoneal disease.
- Description of CRS and HIPEC principles and techniques.
- Analysis of institutional data from 345 patients with PMP or appendix cancer.
Main Results:
- CRS and HIPEC are crucial for achieving complete cytoreduction and improving survival outcomes.
- Long-term survival is achievable in the majority of patients with optimal management.
- Appendiceal neoplasms, though rare, can lead to substantial morbidity and mortality.
Conclusions:
- Optimal management, including complete cytoreduction and HIPEC, is key for long-term survival in patients with PMP and appendix cancer.
- Appendiceal neoplasms require careful management to mitigate significant morbidity and mortality.
- Emerging mucolytic therapies show promise for future treatment strategies.
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