Introduction
Being diagnosed with pseudomyxoma peritonei or appendix cancer is often very confusing, because these are rare diseases that many people have never heard of.
It helps to know from the start that in many cases the prognosis can be very good, especially when the disease is low grade and is treated properly.
What is pseudomyxoma peritonei?
The appendix is a small organ at the beginning of the large intestine. Some tumors of the appendix produce mucin, a jelly-like substance that can build up inside the abdomen.
When this mucin and the tumor cells spread across the peritoneum, we call it pseudomyxoma peritonei.
Reassuring fact
Although it may sound alarming, pseudomyxoma peritonei does not behave like other cancers. In many cases it grows slowly and stays within the abdomen for long periods of time.
Why is the grade of the disease so important?
One of the most important aspects of pseudomyxoma peritonei and appendix cancer is the grade of the disease, which is determined by examining the samples under the microscope.
Put simply, we can talk about:
Low grade
- Slow growth
- Less aggressive
- Generally excellent prognosis with appropriate treatment
High grade
- More aggressive behavior
- Higher risk of relapse
- Need for more intensive treatment
Knowing the grade makes it possible to tailor treatment and give more accurate information about the prognosis.
What symptoms can it cause?
Symptoms usually appear slowly and gradually. The most common are:
- Increase in the size of the abdomen
- Feeling of bloating or pressure
- Mild abdominal pain or discomfort
- Changes in bowel habits
- In some cases, few or no symptoms for a long time
Many people are diagnosed by chance, after surgery or an imaging test.
What does my treatment involve?
The main treatment for pseudomyxoma peritonei and for appendix cancer that has spread to the peritoneum is surgery at specialized centers.
The aim is to:
- Remove as much mucin and visible disease as possible
- Prevent the disease from progressing
- Improve quality of life and life expectancy
Important note
Chemotherapy given through a vein plays a limited role in many of these diseases, especially in low-grade cases. That is why surgery is the main treatment.
Surgery and HIPEC treatment
Surgery aims to clear the abdomen of mucin and visible tumor. In many cases this means removing:
- The appendix
- Parts of the peritoneum
- Segments of the intestine
- The omentum (a layer of abdominal fat)
- Sometimes, other affected organs
Although it can be an extensive operation, in well-selected patients it can give very long-lasting results.
HIPEC
In many cases, surgery is completed with HIPEC, which consists of giving heated chemotherapy inside the abdomen during the operation.
HIPEC:
- Acts directly on any microscopic remains
- Allows high concentrations of chemotherapy with less effect on the rest of the body
- Is part of standard treatment for this disease at many specialized centers
How should I prepare for surgery?
Good preparation helps you recover better:
- Stay physically active at a level that suits you
- Look after your diet, with a focus on protein
- Stop smoking if you smoke
- Ask your questions and make sure you understand the procedure
The team will support you throughout this process.
How long will I be in hospital?
The length of stay depends on the extent of the surgery and on how each person recovers.
- Standard surgery: 10-14 days
- Very extensive surgery: It may be somewhat longer
What will my follow-up be like?
Follow-up is necessary and is usually long term:
- Regular check-ups
- Imaging tests
- Symptom monitoring
For many patients with low-grade disease, follow-up allows a long life with good quality.
A message for you
Although pseudomyxoma peritonei and appendix cancer are rare diseases, the grade of the disease has a strong influence on the prognosis. In many cases, especially low-grade cases treated at specialized centers, the prognosis can be excellent.
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