Peritoneal Tumour Service (PMP)
What is PMP?
Pseudomyxoma is a rare, often slow-growing, low grade cancer that frequently starts in the appendix. The tumour does not spread through the blood but stays in the abdomen where it produces large amounts of a jelly-like mucus and increases in size. This can cause problems with moving and breathing and affects the body's ability to absorb food properly.
What causes PMP?
In the majority of cases an adenoma (tumour) is found in the appendix. Like many other tumours, pseudomyxoma can occur in people who lead healthy lifestyles.
Signs and symptoms
The most common symptom, for both women and men, is a slow increase in waist size . The enlarging abdomen puts pressure on the gut and prevents people from eating normally. Despite this, people often notice an increase in body weight.
How is PMP diagnosed?
It is difficult to diagnose since mucus tumour may be present in the digestive system, gallbladder and ovaries It is often found unexpectedly while investigating discomfort or swelling in the abdomen -This is usually during an ultrasound or CT scan or surgery.
Treatment
The treatment will vary depending on the extent that the tumour has spread. It can involve major surgery to remove organs in the abdomen and its lining the peritoneum. If all or most of the tumour is removed, heated chemotherapy drugs can be put in the abdomen, under anaesthetic, to kill any remaining tumour cells that cannot be seen.
Removing the tumour is a lengthy procedure and surgery can last more than 10 hours
Consent to treatment
When the recommended treatment is explained, and questions answered to your satisfaction, you will be asked to sign a consent form.
The benefits of cytoreductive surgery
These will depend on whether the surgeon can remove all the visible disease and give intraperitoneal chemotherapy. The aim would be to cure the disease or reduce the risk of it coming back.
Alternatives to cytoreductive surgery
Alternative treatments will be discussed with you and may include:
Wait and watch:
monitor the situation closely and, if the tumour continues to grow, we may suggest chemotherapy or surgery.
Debulking surgery:
this is when an attempt is made to remove what is causing the problem or may do so in the near future - not to cure the disease but to deal with particular symptoms.
Chemotherapy:
a chemotherapy trial is currently underway for those patients who are unsuitable for cytoreduction. One of the drugs used - Mitomycin C - is already used in theatre after cytoreductive surgery,. The other - Capecitabine comes in a tablet form. These drugs are known to be relatively well tolerated and patients are monitored with regular follow-up including blood tests and CT scans.
If you decide not to have cytoreductive surgery …
… there is a risk that the tumour may continue to grow and any symptoms you may have could get worse. You can discuss what to do next with a member of the pseudomyxoma team.
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