From the Research
Pseudomyxoma peritonei (PMP) should be treated with cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) to achieve the best outcomes in terms of morbidity, mortality, and quality of life. This approach is supported by the most recent and highest quality study available, which highlights the importance of complete macroscopic tumor excision and the use of heated chemotherapy drugs to kill remaining microscopic cancer cells 1. The treatment involves the removal of all visible tumor tissue and affected organs, followed by HIPEC with mitomycin C at 35-42°C for 60-90 minutes.
Key Considerations
- The prognosis varies based on the histological subtype, with low-grade disease having 5-year survival rates of 63-100% after complete cytoreduction, while high-grade disease has poorer outcomes 2.
- Early diagnosis is challenging due to vague symptoms, including abdominal distension, increased waist size, hernia, and in advanced cases, bowel obstruction 3.
- Treatment should be performed at specialized centers with experience in managing this complex condition, as the expertise of the surgical team significantly impacts outcomes 4.
Treatment Approach
- CRS involves the removal of all visible tumor tissue and affected organs, which may include the appendix, parts of the intestine, gallbladder, spleen, ovaries, uterus, and portions of the peritoneum 2.
- HIPEC is performed using heated chemotherapy drugs, commonly mitomycin C, to kill remaining microscopic cancer cells 4.
- The addition of HIPEC to CRS has been shown to improve survival and reduce tumor growth compared to CRS alone 4.
Importance of Specialized Care
- The treatment of PMP requires a multidisciplinary approach and should be performed at specialized centers with experience in managing this complex condition 3.
- The expertise of the surgical team significantly impacts outcomes, and treatment at a specialized center can improve survival rates and quality of life 1.