so what exactly do all those words mean?
Known as PMP, pseudomyxoma peritonei is a rare cancer that generally starts in the appendix.
When you are newly diagnosed with pseudomyxoma peritonei, it can be very confusing, almost bewildering. There are so many expressions to learn and options to understand – we are here to help with that. Our information is written by patients and reviewed by specialists – that gives you an insight from your point of view that you know is correct.
What is Pseudomyxoma Peritonei?
Pseudomyxoma peritonei is pronounced ‘sue-doh-mix-oh-muh pary-ih-ton-nee-i’ and is often abbreviated to PMP. In most cases, it starts in the appendix; more rarely, it can start in other parts of the body such as the bowel, an ovary or the bladder. It affects around two people per million each year of all ages, both men and women.
A tumour develops in the appendix from a polyp which is a small growth on the inner lining of your appendix. At this early stage, the tumour is rarely discovered as it doesn’t cause any symptoms. Left untreated, the tumour grows and eventually bursts through the wall of the appendix into your abdominal cavity where more tumours then develop. These tumours then secrete a jelly-like substance called mucin which accumulates in your abdominal cavity. It is this build up of mucin that causes symptoms, the abdomen swells leading to the characteristic ‘jelly belly’ and vital organs are compressed.
There are three common classifications of PMP:
This low grade malignancy is a deadly disease without proper treatment.
High grade may also involve “signet ring cells” which are more aggressive cancer cells and can make the disease more difficult to treat.
This is a form of the disease which falls between the two described above.
PMP isn’t like other types of cancer as it seldom spreads through the bloodstream or lymphatic system. It stays contained within the abdomen where it spreads along the inner surfaces, spread by the mucin. It is not currently known what causes PMP. The name ‘pseudomyxoma peritonei’ means “false mucinous tumour of the peritoneum”; the peritoneum is another name for your abdominal cavity.
Spotting the symptoms
PMP symptoms usually take a while to appear; some patients don’t show any symptoms for several years. Symptoms of PMP vary widely between patients so it’s hard to say what to look for. As the disease progresses, many patients report:
• Abdominal swelling or an increase in abdominal girth
• Changes in bowel habit
• Loss of appetite
• Pelvic or abdominal pain
• General fatigue
• Bowel irritation
These symptoms can be caused by lots of medical conditions other than PMP. However, due to the nature of this cancer, it’s vital to rule out PMP as a cause. Any unexplained symptoms should always be discussed with your doctor.
Your appendix is on your right-hand side, just inside your hip and is a small tube, shaped like a worm. It’s about 10cm long, a bit longer than your finger. It’s attached to your large intestine (your colon) where it meets with your small intestine. It is closed at one end and open where it connects to your intestines. Your appendix has a lining that produces mucus as does your small intestine. It can also contract like your small intestine, though unlike your intestines, food does not pass through it – there is no where for it to go.
Your body produces mucus to protect the lining of the tubes in your body, including the intestines and appendix. This mucus is made up of mucin and salts in water. The mucus is produced by a thin layer of epithelial cells that line the inside of your appendix and intestine.
Until recently, it was thought that the appendix had no purpose – that it was a remnant that we had used in earlier times. Now it is thought to play a part in the immune system although it isn’t fully understood what that role is. Your appendix is attached to your caecum, part of your colon. As a result, appendix cancer is considered a colorectal cancer although the standard of care for treatment is different to other colorectal cancers. Colorectal cancers are also part of a larger group of cancers of gastrointestinal tract, or GI, cancers.
Epithelial cells make up one of four tissue types in your body (the others are connective tissue, muscle tissue and nervous tissue). As well as lining the major cavities of your body and forming the structure of your lungs, epithelial cells line most organs including your stomach and you small intestine. You also have them inside your nose – you will be quite familiar with their ability to produce mucus if you have ever suffered from the common cold!
Names are very confusing – the small intestine is sometimes called the small bowel. The large intestine is sometimes called the large bowel or the colon.
What treatment is available?
Treatment for mucinous tumors that have spread into the abdomen, either low grade or high grade, is cytoreductive (debulking) surgery to remove the mucin and tumour implants in the abdominal cavity. This is followed by intraoperative hyperthermic peritoneal chemotherapy (HIPEC). Early postoperative intraperitoneal chemotherapy (EPIC), in which chemotherapy liquid is put into the abdomen using a port or tube soon after the surgery is over, may also be used. The whole procedure is also called “the Sugarbaker technique”, after Dr Paul Sugarbaker.
That’s a lot of terms! Cytoreductive surgery is an operation that removes the tumours that have seeded around the abdominal cavity. It is a very long operation – usually between 10 and 14 hours. The surgeons work to remove all visible tumours in your abdominal cavity, that’s down to 2mm.
HIPEC is liquid chemotherapy solution that is poured directly into the abdominal cavity at the end of the operation. The aim of this is to kill off any remaining cancer cells after the operation to prevent them from growing new tumours. Chemotherapy solution is a liquid chemical solution that kills cancer cells. It is usually heated to about 42C (your body temperature is usually about 37C). When the chemotherapy solution is heated, it is thought to be more effective in killing cancer cells.
The liquid chemotherapy can reach more places in the abdominal cavity while it is still open. It is swished around and circulated inside you to reach areas where those cancer cells that are not visible to the naked eye are hiding. This is done for about 90 minutes (the time can be different). It is drained off and then you can be closed up.
Just in case any of those cancer cells are still hiding in there, you may be given EPIC for several days in a row after your operation. Using a port into your abdominal cavity, the liquid chemotherapy is poured in and then drained out over a 24 hour period.
Why can’t I just have my appendix out?
You wouldn’t usually have any symptoms until the tumour has broken through the wall of the appendix and released mucinous cells into your abdominal cavity. Just having your appendix out at that stage wouldn’t remove those cells and tumours would continue to develop, mucin would continue to be produced and PMP would develop.
For a low grade tumour that hasn’t ruptured and that is confined to the appendix, treatment would be removal of the appendix. When the appendix is removed, it should be contained in a plastic bag to avoid mucin and tumour cells spilling into the abdominal cavity.
For a high grade tumour that hasn’t ruptured, treatment would be an appendectomy and right hemicolectomy (removal of the appendix and up to half of the right side of the colon).
Where can I go for treatment?
In the UK, there’s two hospitals which are recommended by the National Institute for Clinical Excellence (NICE) for the treatment of PMP. These are the Basingstoke and North Hampshire Hospital and the Christie Hospital in Manchester. Both of these hospitals have an NHS Peritoneal Malignancy/Tumour team specialising in PMP. Both also see patients privately in separate clinics.
The referral process to either Basingstoke or to the Christie depends on which part of the UK you live in. For more details, and the contact details for both Basingstoke and the Christie, please visit our UK specialists page.
We do hold information for other countries and we have this under constant monitoring to keep it as up to date as we can.
If you need some help, get in touch. Pseudomyxoma Survivor doesn’t endorse any particular surgeon, specialist or cancer treatment centre. We simply share practical information and national guidelines.
What do you call that?
You may hear lots of terms, unfamiliar words which are difficult to remember. There is so much to take in and you won’t always have time to think to ask the right question. Our glossary is a place where you can look up exactly what something means.
This is original information written by the editorial website team.