Pseudomyxoma peritonei (PMP)
Choose a type
What is pseudomyxoma peritonei (PMP)?
Pseudomyxoma peritonei (PMP) is a rare cancer. It usually starts in the appendix. But it can start in:
Position of appendix and other organs in bowel
The tumour usually grows slowly. But as it grows, it can spread from where it started into other parts of the tummy area (abdomen).
Unlike other cancer types, PMP rarely spreads through the bloodstream or lymphatic system. Instead, PMP cells attach to the layer of tissue that lines the inside of the abdomen. This is called the peritoneum. The peritoneum covers and protects the organs in the abdomen, such as the bowel. It also makes gaps around the organs. These gaps are called the peritoneal space.
PMP cells make a jelly-like substance called mucin. The mucin collects in the peritoneum. This is why PMP is sometimes called ‘jelly belly’.
Side view of ovary, peritoneal space and peritoneum in female body
PMP usually stays within the peritoneal space. But eventually the build-up of mucin puts pressure on the bowel and other organs.
Related pages
Symptoms of PMP
PMP may not cause any symptoms in its early stage. Symptoms of PMP include:
- a gradual increase in waist size
- a hernia (a bulge on the tummy wall)
- loss of appetite
- unexplained weight gain
- tummy pain (abdominal pain)
- pelvic pain
- a change in bowel habit – for example, diarrhoea, constipation or leaking (incontinence).
Related pages
What causes PMP?
Diagnosis of PMP
PMP is rare, and it can be difficult to diagnose. You may have some symptoms, but these can be caused by many other medical conditions. Your GP may arrange tests in a hospital to find out what is causing your symptoms. PMP may be found during abdominal surgery for other medical conditions.
The following hospital tests may be used to diagnose PMP:
-
CT scan
A CT scan uses x-rays to build a three-dimensional (3D) picture of the inside of the body.
-
Ultrasound scan
An ultrasound scan uses sound waves to build up a picture of the internal organs onto a computer screen.
-
Drainage of fluid from the abdomen (peritoneal aspiration)
A doctor may take a sample of fluid from the tummy so that it can be tested. The doctor will numb the skin over the area first with a local anaesthetic. They then pass a needle through the skin into the peritoneal space. They use the needle to draw some of the fluid into a syringe.
-
CT-guided biopsy or ultrasound-guided biopsy
You may also have a biopsy using a CT scan to help find the abnormal area. A biopsy is when a doctor removes a small piece of tissue or cells. Another doctor called a pathologist then examines the tissue or cells under a microscope to look for signs of cancer.
You will have a local anaesthetic to numb the area before the biopsy starts. A doctor then passes a needle through the skin. They use an ultrasound or a CT scan to check the needle is in the right place. The needle has a tip that can cut out a small piece of tissue.
-
Surgery
You may have a biopsy taken during keyhole surgery (laparoscopy). Before surgery, you may have a general anaesthetic. Or you may have a local anaesthetic and a drug to make you feel relaxed and sleepy.
During surgery, the surgeon makes a small cut in your tummy. They then use a special instrument called a laparoscope to look around inside your tummy. A laparoscope is a thin tube with an eyepiece at 1 end and a light and camera at the other end. The surgeon then uses another instrument to take a small piece of tissue.
Waiting for test results can be a difficult time. We have more information that can help.
Related pages
Treatment for PMP
A team of specialists will meet to discuss the best possible treatment for you. This is called a multidisciplinary team (MDT).
Your doctor or cancer specialist or nurse will explain the different treatments and their side effects. They will also talk to you about things to consider when making treatment decisions.
The main treatments for PMP are:
- surgery
- heated chemotherapy given directly into the peritoneum. Chemotherapy uses anti-cancer (cytotoxic) drugs to destroy cancer cells.
Heated chemotherapy is also called hyperthermic intraperitoneal chemotherapy (HIPEC).
Your treatment may depend on how far the PMP has spread and your general health.
Cytoreductive surgery with HIPEC
You may be offered surgery to remove as much of the PMP as possible. This is called cytoreductive surgery. You will then have a heated chemotherapy treatment directly into the peritoneal space. This is called hyperthermic intraperitoneal chemotherapy (HIPEC). It washes out any remaining PMP cells.
Having cytoreductive surgery with HIPEC is also called the Sugarbaker technique, after the surgeon who developed it. It is major surgery, so it is important to discuss with specialist doctors whether it is suitable for you.
The aim of this treatment is to remove as much of the PMP as possible and reduce the risk of it coming back.
During the operation, your surgeon may also remove:
- the gallbladder
- parts of the peritoneum, including the omentum
- the spleen
- in some cases, part of the bowel
- in some cases, the womb (uterus) and ovaries.
When the surgeon has removed all signs of PMP, they put a heated chemotherapy liquid into the peritoneal space. The aim of this treatment is to kill any cancer cells that are left behind. After 60 to 90 minutes, the chemotherapy liquid is drained from the tummy and the surgeon finishes the operation.
Where will I have my treatment?
Only 2 centres in the UK offer specialist surgical treatment for PMP. This is because it is a rare cancer.
The 2 centres that do cytoreductive surgery with HIPEC are:
If you have a long distance to travel to either of these centres, you may need to stay in hospital for longer.
If you are having chemotherapy into a vein (intravenously), you can have it at your local cancer treatment centre.
Before surgery
Before your operation your surgeon and specialist nurse will talk you about the operation They will tell you what to expect and how you can prepare for surgery. We have more information about preparing for surgery.
After surgery
After surgery, you will stay in hospital for about 2 to 3 weeks. For the first few days you will be in a critical care unit. The nurses will help you to get up and to move around. You will have different tubes such as a drip or intravenous infusion to give you fluids until you are able to eat and drink normally. You will also have painkillers which may be given into a vein to begin with.
We have more information about what happens after surgery.
If you need a stoma
If you have had part of the bowel removed, your surgeon may create a stoma as part of the operation. A stoma is an opening that is made through the tummy wall (abdominal wall). It connects the bowel to the surface of the tummy.
Having a stoma means stools (poo) will not pass out of the back passage (rectum) in the usual way. Instead they will pass out of the stoma, into a disposable bag you wear over the stoma. If you are going to have a stoma, you will meet a stoma care nurse before your operation. They will explain what is involved and give you a lot of support.
The stoma may be temporary. This means it can be reversed when you have fully recovered from surgery. This can take 3 to 6 months. Some stomas are permanent.
A colostomy and colostomy bag
Fertility after cytoreductive surgery with HIPEC
Cytoreductive surgery with HIPEC treatment may affect your fertility.
If you have your womb and ovaries removed (hysterectomy and bilateral oophorectomy), you will not be able to get pregnant.
If you are hoping to get pregnant in the future, you should discuss this with your consultant or clinical nurse specialist (CNS). They can talk to you about possible ways to preserve your fertility and can refer you to a fertility expert.
Some cancer treatments can affect whether you can get pregnant or make someone pregnant. Before cancer treatment, it may be possible to store sperm or eggs so that they can be used later. We have more information about fertility.
Chemotherapy
If you cannot have surgery, you may have chemotherapy. This aims to slow the growth of PMP and reduce symptoms. You have chemotherapy as tablets or as a drip (infusion) into a vein.
Watchful waiting
If the PMP is small or growing slowly, you may not need to start treatment straight away.
Your doctor may suggest watchful waiting. Sometimes this is called active surveillance.
This means 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.
Clinical trials
You may have some treatments as part of a clinical trial. Because PMP is rare, there may not always be a trial in progress. Your doctor or nurse can tell you more about this.
After PMP treatment
Follow-up after treatment for PMP
You will have regular check-ups during and after your treatment. Tell your doctor or specialist nurse as soon as possible if you have any problems or notice new symptoms between appointments.
We have more information about follow-up care after treatment.
Side effects after PMP treatment
Sex life
Body image
Well-being and recovery
It can take time to recover after PMP treatment. Some days you may feel better than others.
Looking after yourself can help speed up your recovery. Even small changes may improve your well-being.
Even if you already have a healthy lifestyle, you may choose to make some positive changes after treatment. We have more information on leading a healthy lifestyle after treatment.
It is important to know where to get support or information if you need it. People often need support even some time after PMP treatment. But sometimes it is difficult to know who to ask for help. To find support:
- ask your GP or someone from your cancer team for advice about support in your area
- search cancercaremap.org to find cancer support services near you
- call us free on 0808 808 00 00 or talk to us talk to us online - our cancer information and support specialists can offer guidance and help you find what you need.
Support after PMP
Some people with a rare type of cancer may find it helpful to talk to someone with the same condition. Your cancer doctor or specialist nurse may be able to put you in touch with someone who has PMP and is happy to speak to others. The support network Pseudomyxoma Survivor can also help with this.
The Christie and Basingstoke hospitals each hold annual support days specifically for PMP patients and their relatives. Your specialist nurse at the Christie or Basingstoke can tell you more about this. They will also offer you advice and support.
Macmillan is also here to support you. If you would like to talk, you can:
- call the Macmillan Support Line for free on 0808 808 00 00
- chat to our specialists online
- visit our PMP forum to talk with people who have been affected by PMP, share your experience, and ask an expert your questions
- visit our Ileostomy, colostomy and stoma support forum to talk to people who have an ileostomy or colostomy, or are living with or after a stoma.
Other organisations who offer information and support include:
About our information
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.
-
References
Below is a sample of the sources used in our pseudomyxoma peritonei (PMP) information. If you would like more information about the sources we use, please contact us at
informationproductionteam@macmillan.org.uk
K Govaerts, R J Lurvink, IHJT De Hingh et al. Appendiceal tumours and pseudomyxoma peritonei: Literature review with PSOGI/EURACAN clinical practice guidelines for diagnosis and treatment. 2020. European Journal of Surgical Oncology. Available from: www.pubmed.ncbi.nlm.nih.gov/32199769 [accessed January 2023].
YL Lin, DZ Xu, XB Li et al. Consensuses and controversies on pseudomyxoma peritonei: a review of the published consensus statements and guidelines. 2021. Orphanet Journal of Rare Diseases 16, 85 (2021). Available from: https://ojrd.biomedcentral.com/articles/10.1186/s13023-021-01723-6 [accessed January 2023].
Date reviewed
Our cancer information meets the PIF TICK quality mark.
This means it is easy to use, up-to-date and based on the latest evidence. Learn more about how we produce our information.
The language we use
We want everyone affected by cancer to feel our information is written for them.
We want our information to be as clear as possible. To do this, we try to:
- use plain English
- explain medical words
- use short sentences
- use illustrations to explain text
- structure the information clearly
- make sure important points are clear.
We use gender-inclusive language and talk to our readers as ‘you’ so that everyone feels included. Where clinically necessary we use the terms ‘men’ and ‘women’ or ‘male’ and ‘female’. For example, we do so when talking about parts of the body or mentioning statistics or research about who is affected.
You can read more about how we produce our information here.
How we can help