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Macmillan and Cancerbackup merged in 2008. Together we provide free, high quality information for people affected by cancer through our publications, website and phone service. Find out more|.
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This information is about a condition that can run in families called HNPCC (hereditary non-polyposis colorectal cancer). People with HNPCC have an increased risk of developing cancer of the large bowel (colorectal cancer|) and some other cancers.
HNPCC is a rare condition that runs in families. Conditions that run in families are known as familial or hereditary|.
The term non-polyposis differentiates it from another condition called FAP (familial adenomatous polyposis)|, which is where hundreds of polyps (small growths) develop in the bowel.
HNPCC is the most common cause of hereditary bowel cancer. Fewer than 5 in 100 (5%) of all bowel cancers are linked to HNPCC. Women with HNPCC also have an increased risk of developing womb (endometrial) cancer|.
There is also a slight increase in risk of developing ovarian cancer|. People with HNPCC also have an increased risk of stomach|, pancreas|, biliary| and bladder| cancers.
Knowing about risk and having regular screening may help prevent some cancers and detect others in the early stages when they're curable.
HNPCC is sometimes known as Lynch Syndrome after the doctor who discovered it.
Our genes| carry the information that is passed on (inherited) from our parents. Genes determine things like the colour of our eyes and affect the way our bodies grow, work and look.
HNPCC is caused by a fault in one of the genes known as the 'mismatch repair' gene. These particular genes normally work to help prevent you getting cancer.
HNPCC may be suspected in families with close blood relatives who have developed bowel, womb and ovarian cancer over several generations. They may have inherited a faulty copy of one of the mismatch repair genes.
Three of the mismatch repair genes (known as MLH1, MSH2 and MSH6) are responsible for most cases of HNPCC. So, if a person inherits a faulty copy of one of these genes, it increases their risk of developing bowel cancer and the other types of cancer we've already mentioned.
We have two copies of each gene – one from each of our parents. If someone has HNPCC it means they have a healthy gene but also one that's faulty.
If that person has a child there is a fifty–fifty chance that they will pass on the faulty gene (only one copy of a gene is passed on from each parent).
HNPCC is more likely if there are lots of cases of bowel and womb cancer on one side of the family that were diagnosed at an early age. However, not everyone with HNPCC has a family history of it. This is because some people may be the first in their family to get it.
HNPCC may be suspected if:
If you're worried about cancer in your family, speak to your doctor who can refer you to a family cancer clinic.
HNPCC itself doesn't cause any symptoms. It's an inherited syndrome that means a person has a higher risk of developing bowel and womb cancer.
Sometimes the first sign that a person has HNPCC is when the symptoms of bowel or womb cancer develop. This generally happens at a younger age than people whose cancers aren't due to an inherited faulty gene. And there's usually a history of these cancers in the family.
Bowel cancer that doesn't run in families usually develops in people over 50, but with HNPCC, bowel cancer usually occurs between the ages of 40 and 50 or younger.
Being aware of your normal bowel habit is important, particularly if you have or think you may have HNPCC.
If you have any of the following symptoms it's important to get them checked out by a doctor:
It's also important to be aware of the symptoms of womb cancer if you have or think you may have HNPCC. Any of the following symptoms should be checked out by a doctor:
All these symptoms can be caused by conditions other than cancer, but it's always important to get them checked out by your doctor.
It's possible to find out if someone has HNPCC by doing a genetic test|. This test is first carried out on the family member who has had cancer. If the faulty HNPCC gene is found in that person, then close family members (not affected by cancer) can be tested (if they wish) to see if they have inherited it.
If a person has a suspected HNPCC-type cancer, a special test may be done on a tissue sample taken from the tumour before genetic testing. If this is positive, genetic testing is offered.
Genetic testing can only be carried out if a person is willing to have it. All that's needed is a blood sample, but it can take a while (up to a year) to get results as the genes are large and the faulty gene may be difficult to find.
Once the faulty gene has been found, other family members can then be tested for the same faulty gene.
Before genetic testing, a person will have counselling to help them understand the possible results and what they may mean for them and their family.
Sometimes the faulty gene can't be found in the person with the HNPCC-type of cancer (because it's not HNPCC or there's a fault in the gene that research hasn't yet identified). If no gene change is found other family members can't be tested. However, based on their family history, they can still have regular bowel tests and womb checks (in women) to reduce their cancer risk.
What the test results mean:
Knowing your risk of cancer means you can have regular tests (screening). Bowel cancers can be curable when they're picked up early.
If a person is found to have inherited the faulty gene, they'll usually be advised to have regular bowel screening from a young age. This may begin at the age of 25 or five years earlier than the age your youngest affected relative developed bowel cancer. Some women may also be offered screening for womb cancer.
If the faulty gene wasn't found in the person with HNPCC-type cancer, it's important that you still have screening as you may still be at risk of cancer. This is the same if you decided against genetic testing or couldn't have it because a family member with HNPCC-type cancer didn't want a test.
A colonoscopy is used to examine the bowel and diagnose any cancers early on when they're curable.
The test| is usually carried out in the hospital outpatient department and takes about an hour. Your bowel has to be completely empty for the test. This means you'll have to follow a special diet for a few days beforehand. On the day before your colonoscopy, you'll be asked to take medicine (a laxative) to empty your bowel. Your hospital will give you instructions about preparing for a colonoscopy. Just before the test, you will be given a drug to make you feel more relaxed (sedative). This may be given as an injection into a vein (intravenously).
Once you're lying comfortably on your side, the doctor or nurse will gently pass a flexible tube (a colonoscope) into your back passage. A colonoscope is a long, thin, flexible tube that has a tiny light and a camera on the end of it. It can easily pass around curves, so most of the large bowel can be examined. During the test, photographs and samples of tissue (biopsies) may be taken. Any polyps (small growths) can also be removed before they develop into a cancer.
Afterwards, you will need somebody to go home with you and stay with you for 24 hours until the effects of the sedative wear off. It's important not to drive during this time. Colonoscopies are usually repeated every two years.
We don't yet know if womb screening in women with HNPCC is helpful. Some women may be offered it, but it's not available everywhere.
The womb can be screened using a procedure called a hysteroscopy or by using a vaginal ultrasound. Your doctor or nurse will explain which test you will have. During a hysteroscopy a thin, flexible tube with a light at the end will be used to look inside the uterus. A vaginal ultrasound scan involves putting a small device that makes sound waves into the vagina. The sound waves are then converted into a picture by a computer.
We don't know if ovarian screening helps pick up ovarian cancer at an earlier stage. Occasionally some women may be offered it or they may have it done as part of a research trial|.
The risk of developing ovarian cancer if you have HNPCC is much lower than your risk of bowel or womb cancer. Screening can involve a blood test, a vaginal ultrasound or both. The blood test checks the levels of a protein called CA125.
If you develop bowel cancer, it's likely to be picked up early through having regular colonoscopies. Any HNPCC-type cancer is treated in the standard way for that type of cancer.
Treatment for bowel cancer will usually involve surgery| to remove the cancer. Further treatment with chemotherapy| may be needed, depending on the stage of the cancer.
Treatment for womb cancer will usually involve removing the womb (hysterectomy) and the ovaries|. Radiotherapy| may also be given.
Occasionally with HNPCC, it's recommended that the whole of the large bowel is removed as a preventative measure. This is known as risk-reducing surgery. It's usually only recommended where the risk of developing cancer is thought to be very high or if a person with HNPCC develops more than one bowel cancer.
Some women (when they've completed their families) may choose to have their womb and ovaries removed to reduce their risk of womb and ovarian cancer.
Knowing that you have HNPCC or are at risk of it can be very difficult to cope with. The uncertainty of not knowing| if you will develop cancer isn't easy to deal with, but it's important to remember that bowel cancer can be found early and cured. You may have concerns about genetic testing, screening or whether you should have risk-reducing surgery.
It's important to talk these concerns over with the doctors and nurses caring for you. They'll be happy to answer any questions you have.
You may have many different emotions|, including anxiety and fear. These are all normal reactions and are part of the process that many people go through in trying to come to terms with their condition.
Many people find it helpful to talk things over with their doctor or nurse. Close friends and family members can also offer support.
For answers, support or just a chat, call the Macmillan Support Line free (Monday to Friday, 9am-8pm)
If you have any questions about cancer, need support or just want someone to talk to, ask Macmillan.