Lynch Syndrome UK

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The Cancer Research UK website has very good information. It is in the section called “small bowel cancer.”


Small bowel cancers are more common in people with Lynch syndrome than in those that do not have it. It seems to affect more people with MLH1, MSH2 and Epcam than people with MSH6 and PMS2.


The small bowel consists of the duodenum which is just below the stomach, the jejunum which follows on from that and the small intestines which are a mass of coiled narrow tubes before the food enters into the colon.

The small bowel contains food which is fairly liquid and it absorbs nutriments to sustain the body and also releases enzymes and vitamins to absorb and digest food.


Small bowel cancers in Lynch syndrome are not as common as colon and rectal cancers but they are difficult to diagnose and frequently do not produce tell-tale symptoms. That means they can grow to a later stage without being discovered.

In Lynch syndrome, most of the small bowel cancers (at least 50% of them) occur in the duodenum.  The rest are spread through the jejunum and small intestines, fewer as you go down.


40% of small bowel cancers are adenocarcinomas.




“How many cases of small bowel cancer occur each year in the UK?”



“How many of those are due to Lynch syndrome?”

Probably 10% of those


“What is the risk of getting small bowel cancer for people with Lynch syndrome?”

The risk of getting small bowel cancer for people with MLH1 and MSH2 is approximately 5% by the age of 70 years. That means that out of 100 people with those LS gene mutations, 5 will get small bowel cancer by that age. This is not regarded as high risk and therefore in the UK, screening of the small bowel is not routinely done.



Family history


There is no published evidence that Lynch syndrome small bowel cancer clusters in families so risk should not be increased just because an LS relative has had this cancer. But there may be other sporadic (or non LS gene mutations) that influence the formation of cancers and some families do have screening of relatives. Some do have some form of upper gastrointestinal tract (GIT) screening and it may reassure relatives to have at least some part of the small bowel looked at regularly. (see screening)



(With thanks to Cancer Help UK for pictures)


Glossary …..Motions = stools/poo/faeces


The duodenum and jejunum are small tubes through which a fairly mushy collection of food and liquid passes through. A cancer there causes symptoms of blockage, pain behind the bottom of the breast bone, bleeding into the small bowel and blockage.

If the cancer occurs in the duodenum at or close to the head of the pancreas, that will produce additional symptoms cause by blockage of the hepatic and pancreatic ducts. Those are the entry points for various enzymes and liquids – bile from the liver and gall bladder and other enzymes such as amylase from the pancreas. Blockage there might cause, jaundice, indigestion, carbohydrate intolerance and intermittent pain that is worsened by eating. Diarrhoea will often be caused by food malabsorption – producing fatty, smelly motions, pale if there is a halt of bile into the gut but dark black if there is bleeding.

Symptoms in small bowel cancer can include any or all of the following:-


Indigestion – vague pain behind the breast bone or just below it, going through to back. Might spread round on the LHS. May be worse with eating and volume of food may have to be reduced.

Pain in epigastrium – the “pit of the stomach” may end up as continuous, not relieved by medications. Can end up as relentless back pain at level of the breasts


Nausea and vomiting – starting as feelings of sickness will eventually increase to being unable to keep food down. Vomiting immediately after food occurs later if the lumen of the small bowel becomes occluded.

Diarrhoea can be an early feature due to food malabsorption if cancer is in the duodenum at site of pancreatic and hepatic ducts. Stools are loose, smelly, pale or dark black from altered blood.

Jaundice – caused by blockage of the hepatic ducts – dark yellow urine, yellow skin and eyes, pale motions.

Paleness and tiredness due to anaemia is as a result of bleeding into the small bowel

Weight loss – unexplained by dieting



Spread and staging in small bowel cancer in Lynch syndrome


All of the small bowel is very thin walled so cancers do tend to break through into surrounding tissues quite quickly. Lynch syndrome cancers tend to have better prognosis than ordinary “sporadic” cancers until there is spread elsewhere. If it goes into the blood stream it will pass to the local gastrointestinal lymph nodes and the liver.

Lynch syndrome cancers tend to have a better prognosis (at least 10% better by 10 years) because they produce a particular level of “immune response” such a higher level of  T-cell lymphocyte infiltration than usual.




The lower the stage the better. Treatments vary from stage to stage so don’t worry if picture is rather mixed at first.

Macmillan has a good description of staging in small bowel cancer and other information…





Gastoscopy – endoscope into the stomach

Duodenoscopy – endoscope into the duodenum

CT scan

MRI scan

Capsule video-endoscopy – a capsule with a video on it, swallowed and x ray pictures recorded in real time






Treatment for small bowel cancer invariably means surgery.  If additional treatment is required that will usually be chemotherapy – either before your surgery to shrink the cancer or afterwards to mop all the lumps of cancer cells that have not been identified at surgery or afterwards as a means of mopping up all those and the cancers cells that might have spread through the blood stream. Make sure that you ask whether Lynch syndrome specific chemotherapy will help – it will depend on the histology of your cancer.

Radiotherapy might be used later in the illness to shrink localized areas of cancer.




Surgery is horrible and you will feel like you’ve been put through a mangle but getting rid of that awful lump of cancer will make you feel 100 times better – you only have to recover now. If you have had a chunk of your small bowel taken out plus maybe some of your pancreas, your gall bladder and a bit of your liver – you will not want to be prodded and poked. Try and sleep 23 hours a day, keep visitors to a minimum. Do not expect to get over this in a couple of days – it might be a few weeks and you might be in hospital for weeks not days . .  You might be on ITU for a short while.

First battle is pain – make sure you get as much pain relief as you need. Don’t be brave. Be honest with the nurses and get as much sleep as you can.  The scar outside looks impressive – the scars inside are ten times worse so be sensible, get up when you’re told and lie back when they say so!


Eating and getting your bowels going will be a hurdle. There is no other way round it than to try small amounts of low fibre food and wait for it to trickle through. Hospital liquidized food is ghastly – liquidized carrots and mash and liquidized meat looks dreadful and is completely unappetizing with very few calories. In that case prepare beforehand and get a cupboard full of all your favourite “smooth” foods. That might be Angel Delight, crème caramel, ice cream, thick butternut squash soup, - all the things that are really tasty, smooth and also full of calories. Just beware that fat absoption might not be good to begin with. See if they can send a dietician down to see you.


Surgery on the small bowel is different to colon surgery in the fact that it is (relatively) easy to remove a section of the colon without it feeling like major surgery. It is fairly easy to recover from it. But surgery on the small bowel is taking out a fundamental section of the GIT. To suddenly do without the drainage from the sink in your kitchen plus bits of the sink itself, the food grinder and some of draining board itself will lead to chaos if you try to wash up!


Take it slowly, don’t rush stages of eating different food and remember that it will take many months for you to get back to normal. Sometimes you will be constipated, then you will have diarrhoea and sometimes you will have pain. Always get it checked out by your doctor but you have had some serious plumbing surgery – but it will sort itself out in the end. Weight yourself weekly and keep a food diary if you don’t seem to be tolerating foods well.


Don’t drink alcohol after your surgery until the doctors say it is ok (your liver and pancreas want a holiday) and keep all visitors away who have coughs and colds – you do not want any kind of virus after surgery and during chemo.

Do not let anyone smoke in your house – you want a 100% oxygen rich environment for your recovery.


DO NOT LET PEOPLE RUSH YOU! I’d like to write this in letters 50 x big. Show this to all your friends and relatives …. People think that when you’ve had your surgery and have got home that when the scar heals you are “on your road to recovery”. You’re not! Being discharged is only the beginning – you need 12 months of convalescence and special treatment so do not entertain the idea of work, exercise, big meals, parties, booze, car trips  etc until you are over the finishing line and passed as fit by your doctor.


At times you will be at your wits end, particularly if you have chemotherapy after your surgery, which is common with small bowel cancer – you will find it difficult to stop feeling sick and to get your weight back to normal. Some days you will feel like crying, at others you will want to give up…Be patient and persevere. If the surgeon has removed 95% of the cancer, just stick with it through the first 12 months after your surgery. Then have a great big party!!!




Don’t read survival figures for small bowel cancer!! They are mostly out of date and don’t apply to Lynch syndrome. They can look extremely gloomy so regard your treatment as being successful from the word go. Depression is the enemy of your immune system and you should be positive but also realistic. Get your treatment done as soon as possible and concentrate on getting better.




At present the UK does not do routing screening of the small bowel in Lynch syndrome. That is because there is not a satisfactory method that is both safe and effective. The following methods are undertaken by some gastroenterologists in some areas. The intervals are usually 1-2 years.


Gastroscopy and “look through”


Because at least 50% of small bowel cancers in LS are in the duodenum, some LS mutation carriers (most often MLH1 and MSH2) with a family history are offered gastroscopy (looking into the stomach) then pushing the scope through into the duodenum for a “look through”. This will only pick up duodenal cancers but will catch about 50% of small bowel cancers so is a compromise.




Is similar to the above. It just looks at the duodenum but is a better look.


You swallow a special pill – a large pill shaped video cam and carry around a receiver for 24 hours. It then takes a few days for a radiologist to read the pictures.


None of these tests are perfect and some people have had clear results but then a cancer has appeared soon afterwards. (A false negative)


Other people have had false positives – they have been told they have cancer but haven’t got it.

Therefore if you have MLH1 or MSH2 and have a relative who has had a Lynch related small bowel cancer ask to be referred to a gastroenterologist to discuss whether screening is advised and available at your hospital. You might need to be referred to a teaching hospital if you have a Pill-cam.




Take aspirin or be on the CaPP3 trial

Don’t eat a very hot spicy type of diet (as in Korea)

Probably will help to keep alcohol at sensible levels

Don’t smoke

Make sure your Helicobacter pylori status is checked and treated if positive.




If you need any more information, you can contact one of us at Lynch Syndrome UK

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