Pseudomyxoma peritonei (PMP) is a rare cancer that often grows slowly. It usually starts in the appendix. Sometimes it starts in another part of the bowel, the bladder or the ovaries but this is rare. We do not know what causes PMP.
PMP can spread from the appendix (or wherever it started) into the abdomen. PMP makes a jelly-like substance called mucin. The mucin collects in a layer of tissue that lines the inside of the abdomen, called the peritoneum. The peritoneum covers and protects the organs in your abdomen, such as the bowel. It also makes gaps around the organs. These gaps are called the peritoneal space.
It may be many years before you have any symptoms. Eventually the build-up of mucin puts pressure on the bowel and other organs. PMP usually stays within the peritoneal space.
PMP can be difficult to diagnose. You may have some of the symptoms listed above and go for tests. But PMP is often found when having tests or during treatment for other medical conditions. The following tests may be used to diagnose PMP.
Drainage of fluid from the abdomen (peritoneal aspiration)
Your doctor may take a sample of fluid from your tummy to be tested. They will numb the skin over the area first with a local anaesthetic. The doctor will then pass a needle through the skin into the peritoneal space. They will use the needle to draw some of the fluid into a syringe.
You may also have a biopsy. This is when the doctor removes a small piece of tissue or cells. A doctor who specialises in analysing cells (pathologist) examines the tissue or cells under a microscope to look for signs of cancer. A biopsy can be taken in different ways:
CT or ultrasound-guided biopsy
A biopsy may be taken during keyhole surgery (laparoscopy). You have either a general anaesthetic or a local anaesthetic and a drug to make you feel relaxed and sleepy.
The surgeon makes a small cut in your tummy (abdomen). They use a special instrument called a laparoscope to look around inside your tummy. A laparoscope is a thin tube with an eyepiece at one end and a light and camera at the other end. Your doctor then uses another instrument to take a small piece of tissue.
The main treatments for PMP are surgery and chemotherapy. Your treatment may depend on how far the PMP has spread and your general health.
A team of specialists will meet to discuss the best possible treatment for you. This is called a multidisciplinary team (MDT).
Your doctor will explain the different treatments and their side effects. They will also talk to you about the things you should consider when making treatment decisions.
Cytoreductive surgery with HIPEC
Cytoreductive surgery with HIPEC is an operation that aims to remove the PMP. It is also called the Sugarbaker technique, after the surgeon who first developed it.
It is major surgery, so it is important to discuss whether it is suitable for you with specialist doctors. After the surgery, you will stay in a critical care unit for a few days and in hospital for about 2 to 3 weeks. There are only two centres in the UK that offer cytoreductive surgery with HIPEC. If you have a long distance to travel, you may need to stay in hospital a bit longer.
The two specialist centres that do cytoreductive surgery with HIPEC in the UK are:
The aim of the operation is to remove as much of the PMP as possible. This often means removing:
- the gallbladder
- parts of the peritoneum, including the omentum
- the spleen
- in some cases, part of the bowel.
In women, the surgeon may also remove the womb (uterus) and ovaries.
When the surgeon has removed all signs of PMP, they put a heated chemotherapy liquid into the peritoneal space. This is called hyperthermic intraperitoneal chemotherapy (HIPEC).
Chemotherapy uses anti-cancer (cytotoxic) drugs to destroy cancer cells. The aim is to kill any cancer cells that are left behind. After 60 to 90 minutes, the chemotherapy liquid is drained from the abdomen and the surgeon finishes the operation.
Some people will have more chemotherapy given into their peritoneal space during the first 5 days following the operation. A nurse or doctor will give this through tubes put into your abdomen.
Some people will have a stoma as part of the operation. A stoma is an opening that is made through the tummy wall (abdominal wall) and connects to a part of the bowel. Stools (poo) will no longer pass out of the rectum and anus. Instead, it will pass out of the stoma into a disposable bag that is worn over it. The stoma is round or oval and usually looks pink and moist. It has no nerve supply, so it does not hurt.
The stoma may be temporary. This means it can be reversed with a small operation after 3 to 6 months when you have recovered from surgery. Sometimes a stoma may be permanent.
The aim of this surgery is to reduce the symptoms. This treatment will not remove all of the PMP. You may need to have more debulking surgery later.
It is common to have a permanent stoma after debulking surgery. This can help prevent the bowel from becoming obstructed (blocked).
If you cannot have surgery, you may have chemotherapy to slow the growth of PMP and to reduce symptoms. You have chemotherapy as tablets, or as a drip (infusion) into a vein.
You will have CT scans, blood tests and regular check-ups to monitor the PMP and your symptoms. If the PMP begins to cause you problems, your doctor will talk to you about starting treatment.
Everyone has their own way of coping. Some people find it helpful to talk to family or friends. Others get help from people outside their situation. Sometimes it is helpful to share your experiences at a local cancer support group. There is no right or wrong way to cope, but help is there if you need it.
Some people with a rare type of cancer may find it helpful to talk to someone with the same condition. Your specialist doctor may be able to put you in touch with someone who has PMP and is happy to speak to others. There is an organisation called Pseudomyxoma Survivor who can also help with this.
Macmillan is also here to support you. If you would like to talk, you can:
Below is an example of a source used in our pseudomyxoma peritonei information. If you would like more information about the sources we use, please contact us at email@example.com
NICE Interventional procedures guidance [IPG56]. Complete cytoreduction for pseudomyxoma peritonei (Sugarbaker technique). www.nice.org.uk/guidance/ipg56 (accessed May 2019).
This information has been written, revised and edited by Macmillan Cancer Support’s Cancer Information Development team. It has been reviewed by expert medical and health professionals and people living with cancer. It has been approved by Senior Medical Editor, Professor Tim Iveson, Consultant Medical Oncologist.
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