From the Research
Pseudomyxoma peritonei (PMP) is a rare clinical condition that should be managed with a combination of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for optimal outcomes, as supported by the most recent and highest quality evidence 1.
Overview of Pseudomyxoma Peritonei
PMP is characterized by the accumulation of mucinous material within the peritoneal cavity, typically originating from a ruptured appendiceal mucinous neoplasm. This condition progresses slowly, causing symptoms like increasing abdominal girth, digestive difficulties, and eventual compression of vital organs. PMP is classified into low-grade and high-grade variants, with the low-grade form having a better prognosis.
Diagnosis and Treatment
Diagnosis typically involves imaging studies such as CT scans showing characteristic "scalloping" of organ surfaces, followed by histological confirmation.
- The standard treatment approach combines cytoreductive surgery (CRS) to remove all visible disease with hyperthermic intraperitoneal chemotherapy (HIPEC), typically using mitomycin C at 40°C for 90 minutes or oxaliplatin for 30 minutes.
- This aggressive approach offers the best chance for long-term survival, with 5-year survival rates of 70-90% for low-grade disease, as reported in recent studies 2.
- For patients with unresectable PMP, modified FOLFOX6 chemotherapy may be an effective and tolerable treatment option, with a disease control rate of 87.5% and median progression-free survival of 13.0 months 3.
Follow-up and Specialized Care
Follow-up monitoring includes regular tumor marker tests (CEA, CA19-9, CA-125) and imaging studies.
- Due to the rarity and complexity of PMP, treatment at specialized centers with experience in peritoneal surface malignancies is strongly recommended to optimize outcomes and minimize complications, as emphasized in recent reviews 1.
- A multidisciplinary approach and specialized care are crucial for improving outcomes and quality of life for PMP patients.