Pseudomyxoma peritonei

Yes. Pseudomyxoma peritonei can be cured with complete cytoreductive surgery and HIPEC, especially in low-grade tumors. In well-selected cases, 10-year survival exceeds 70%. The condition is that no visible disease remains at the end of surgery, which requires a team with specific experience.

It is almost always caused by a mucinous tumor of the appendix, although it can also come from the ovary, the colon or other organs. When that tumor ruptures, it releases into the abdominal cavity cells that produce mucin and keep making it. That mucin builds up progressively and is what gives the condition its characteristic appearance.

Progressive abdominal swelling, an increase in abdominal girth, a feeling of fullness, loss of appetite, changes in bowel rhythm and general discomfort. These symptoms are non-specific and appear slowly, so many patients wait months for a diagnosis. It is not unusual for it to be discovered during an operation for another reason.

Yes. With a localized recurrence, further cytoreductive surgery can be considered, with the same chances as the first. The condition is again to remove all visible disease. The prognosis depends on the extent of the relapse, the histological grade and the general condition, and some patients are operated on several times over the years.

No. Pseudomyxoma is a very rare disease, with an incidence of about one case per million people per year. Most hospitals see very few, and here the result depends on achieving complete cytoreduction in the first operation. That is why a team with proven experience in peritoneal surgery is advisable.

Its effectiveness is limited: pseudomyxoma responds poorly to systemic chemotherapy, so the treatment of choice is cytoreductive surgery with HIPEC. Chemotherapy may be considered in high-grade cases or when the disease cannot be resected, but it does not replace surgery in low-grade tumors.

It is major surgery, with a risk of bleeding, infection, leakage at the bowel joins and thromboembolic complications. At specialized centers mortality is below 2% and serious complications are uncommon. The difference between a high-volume center and one without that volume shows precisely in these figures.

The hospital stay is usually 10 to 14 days and full recovery takes between 2 and 3 months. The return to normal activity is gradual and depends on the extent of surgery and whether there were bowel resections. Enhanced recovery protocols shorten the stay and reduce complications.

Check-ups with tumor markers (CEA, CA 19-9 and CA 125) and CT every 3 to 6 months during the first years, then yearly check-ups. Follow-up continues for a long time because pseudomyxoma can come back years later and, if detected in time, can again be operated on with curative intent.

At centers with proven experience in surgery for peritoneal carcinomatosis and, specifically, pseudomyxoma. It is worth asking how many cases the team treats each year and what complete cytoreduction rate it achieves. Our institute treats this disease routinely, with a multidisciplinary board and a second opinion service for patients from other centers.