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Pseudomyxoma peritonei (PMP) usually begins as a
slow-growing tumour in the appendix, called a Low-Grade Mucinous Appendiceal
Neoplasm (LAMN). Rarely, PMP starts in other parts of the bowel, ovary or
bladder.
Over time, the tumour produces a jelly-like
substance called mucin. This can cause the appendix to swell up like a balloon.
The tumour can then break through the wall of the appendix and spread tumour
cells into the lining of the tummy (the peritoneum).
The tumour cells and mucin build up in the lining
of the tummy, putting pressure on the bowel and causing symptoms. It can be
many years before symptoms become obvious. Unlike other cancers, PMP rarely
spreads via the lymphatic system or the bloodstream. It usually remains inside
the tummy, spreading along its internal surfaces.
The cause of PMP is unknown.
Most people don't have any symptoms for a long
time. When symptoms occur they may include any of the following:
- slow increase in waist size
- hernia (a swelling on the abdomen)
- loss of appetite
- unexplained weight gain
- abdominal or pelvic pain
- changes in bowel habits
- appendicitis.
Most people with these symptoms won't have PMP,
but it's important to have any symptoms checked by your doctor.
PMP can be difficult to diagnose. It may be found
during investigations into abdominal symptoms, or it may be discovered during
an operation for another problem.
CT (computerised tomography) scan
A CT scan takes a series of x-rays that build up a
three-dimensional picture of the inside of the body. The scan is painless. It
can help to find where the tumour started and check whether it has spread
within the abdomen. It usually takes 10-30 minutes. CT scans use a small amount
of radiation, which is very unlikely to harm you and won't harm anyone you come
into contact with. You will be asked not to eat or drink for at least four
hours before the scan.
You may be given a drink or injection of a dye,
which allows particular areas to be seen more clearly. For a few minutes, this
may make you feel hot all over. If you are allergic to iodine or have asthma
you could have a more serious reaction to the injection, so it's important to
let your doctor know beforehand.
Sometimes the pictures from the CT scan are enough
to make the diagnosis, but sometimes biopsies or an operation are needed to be
sure of the diagnosis of PMP.
The treatment of PMP depends on a number of
factors. These include how far the tumour has spread and your general health.
Some of the standard cancer treatments, such as radiotherapy, aren't suitable
for treating PMP. This is because PMP cells aren't sensitive to radiotherapy
and they are often spread over too large an area for this treatment.
Surgery
You may be offered surgery to treat this kind of cancer. There
are two types of surgery:
- Cytoreductive surgery and hyperthermic
intraperitoneal chemotherapy (HIPEC).
- Debulking surgery.
Cytoreductive surgery and hyperthermic intraperitoneal
chemotherapy (HIPEC)
This may be an option for some people. It‘s an
intensive treatment that aims to remove the tumour to try to cure PMP. It is
also known as the Sugarbaker technique (named after the surgeon who first
developed it). It involves removing the lining of the abdomen or organs such as
the bowel, omentum (fatty tissue in the tummy) and gallbladder. In women, the
womb (uterus) and ovaries may also be removed. About half (50%) of people who
have a Sugarbaker operation will need a stoma (colostomy). Most of the stomas
are temporary and will be reversed after about six months.
Once the surgeon has removed all or most of the
tumour, a heated chemotherapy drug is put in the tummy (hyperthermic
intraperitoneal chemotherapy) for 90 minutes during the operation. The
combination of the chemotherapy drug and heat aims to kill any tumour cells
that are left behind.
This is a major operation and may take up to 10
hours. Afterwards, you’ll be nursed in a critical care unit for several days
and will stay in hospital for about 2 weeks. This operation has potentially
serious complications and the surgeon will discuss these with you.
The National Institute for Health and
Clinical Excellence (NICE)
is an organisation that currently advises doctors on treatments for all types
of illness. It has produced guidelines about this type of surgery with
intraperitoneal chemotherapy. You can read the guidelines on the NICE website.
It's very important to discuss this operation with
specialist doctors, as the Sugarbaker technique is a very complicated procedure
and isn't suitable for everyone. It should only be carried out at a specialist
centre. There are two in the UK:
Debulking surgery
This is done when it’s not possible to have
cytoreductive surgery. It aims to remove as much of the tumour as possible to
reduce the symptoms of the cancer. This may involve removing the omentum (fatty
tissue in the tummy) and part of the bowel. In women, the womb (uterus) and
ovaries may also be removed.
Unfortunately, this surgery will not take away all
the tumour cells and the PMP is likely to grow back. Further debulking
operations may be needed. However, each operation becomes more difficult to do,
with less benefit and more risks of complications each time.
Sometimes, a permanent stoma is needed after
debulking surgery. It can help to prevent the bowel from becoming blocked
(obstructed). Your specialist nurse can give you more information about looking
after a stoma.
Chemotherapy
Chemotherapy can be used to treat PMP. Some
people who can’t have surgery may benefit from chemotherapy. It does not cure
the cancer but can be used to slow it down. Research into other treatments for
PMP is ongoing and advances are being made. Cancer specialists use clinical trials to assess new treatments. You may be
asked to take part in a clinical trial. Your doctor must discuss the treatment
with you so that you have a full understanding of the trial and what it means
to take part.
Watchful waiting
For some people, the risks of treatment may
outweigh the potential benefits, especially as this can be a slow-growing
cancer. If you're in this situation, your specialist may suggest watchful
waiting. This involves being monitored closely with regular check-ups. Only if
the PMP begins to cause you problems will your specialist discuss starting
treatment.
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Content last reviewed: 1 February 2013
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