What are the best treatments for Pseudomyxoma Peritonei?

TL;DR: The gold-standard treatment for Pseudomyxoma Peritonei typically involves cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC). Because Pseudomyxoma Peritonei is a rare and complex condition, treatment must be highly personalized and managed by specialized centers experienced in high-volume peritoneal surface malignancy care.

What are the primary treatments for Pseudomyxoma Peritonei?

For many patients, the primary goal is the surgical removal of all visible tumor deposits within the abdomen, a procedure known as cytoreductive surgery. To address microscopic disease, surgeons often perform HIPEC, where heated chemotherapy (such as mitomycin-C or oxaliplatin) is circulated directly in the abdominal cavity. While surgery is the cornerstone, management of Pseudomyxoma Peritonei also includes:

• Systemic Chemotherapy: Used in cases where surgery is not an option or for patients with specific tumor biology.


• Palliative Care: Focused on managing symptoms like bowel obstruction, pain, and ascites.


• Nutritional Support: Essential for patients managing the gastrointestinal impact of Pseudomyxoma Peritonei.

Which specialists should be on the care team?

Managing Pseudomyxoma Peritonei requires a multidisciplinary approach due to the complexity of the disease. Your care team should ideally include a surgical oncologist specializing in peritoneal surface malignancies, a medical oncologist, a radiologist, and a specialized nutritionist. At DiseaseMaps.org, 110 people with Pseudomyxoma Peritonei have shared their experiences, highlighting the value of connecting with centers that manage a high volume of these specific cases.

How does treatment effectiveness vary?

The success of Pseudomyxoma Peritonei treatment depends heavily on the "completeness of cytoreduction" (CC score) achieved during surgery, the grade of the tumor, and the patient's overall health status. Because Pseudomyxoma Peritonei can range from low-grade indolent disease to more aggressive forms, there is no "one-size-fits-all" protocol. Ongoing clinical trials are currently investigating the role of targeted therapies and immunotherapy for patients who do not respond to traditional surgery.

Next steps

• Consult with a surgical oncologist at a high-volume center specializing in Pseudomyxoma Peritonei.


• Join the community at DiseaseMaps.org to connect with 110 others living with this diagnosis.


• Request a review of your pathology slides by a pathologist who specializes in rare appendiceal or peritoneal tumors.


• Discuss potential clinical trial eligibility with your medical team.

Medical disclaimer: This information is for educational purposes only and does not constitute medical advice; always consult your personal healthcare team for treatment decisions.

References

• NIH Genetic and Rare Diseases Information Center (GARD): Pseudomyxoma peritonei.


• Orphanet (ORPHA:758): Pseudomyxoma peritonei.


• National Cancer Institute (NCI): Peritoneal Surface Malignancies Information.


• PMP Pals Network: Patient-led resources and support for Pseudomyxoma Peritonei.