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The Amsterdam and Bethesda criteria, explained simply | CION Cancer Clinics
The Amsterdam criteria and the Bethesda guidelines are two checklists doctors use to spot families who may have Lynch syndrome. Amsterdam describes a strong family pattern. Bethesda picks out bowel tumours that should be tested. Neither diagnoses Lynch, and both miss many families. This page explains what each asks, how they are used today, and what to do if your family fits. At CION Cancer Clinics, our oncologists plan screening and care for families with an inherited cancer syndrome, explained in plain words.
On this page
- What are the Amsterdam and Bethesda criteria?
- What does each checklist actually ask?
- How does a doctor use these criteria now?
- What do the terms around these criteria mean?
- How do the two checklists compare?
- What this page cannot tell you
- Four things families tell us, and what is actually true
- Common questions about the Amsterdam and Bethesda criteria
The short answer
What are the Amsterdam and Bethesda criteria?
They are two checklists doctors use to spot families who might have Lynch syndrome, the most common inherited cause of bowel cancer. The Amsterdam criteria describe a family pattern that strongly suggests Lynch. The Bethesda guidelines pick out which bowel tumours should be tested for signs of it. Neither one diagnoses Lynch syndrome. Only a genetic test can do that.
Why there are two lists
The Amsterdam criteria came first and were written to define families for research. They are strict, so a family that meets them is very likely to have an inherited cause. The Bethesda guidelines were written later to cast a wider net, so that more people with Lynch would have their tumour checked and be offered testing.
Why they matter less than they used to
Both lists miss many families with Lynch syndrome, especially small ones or those where cancers were never properly recorded. Many guidelines now recommend checking every bowel and womb cancer for signs of Lynch, whatever the family history. The criteria are still useful, but they are no longer the only gate.
Not meeting either checklist does not rule out Lynch syndrome. Meeting one does not confirm it.In plain language
What does each checklist actually ask?
Doctors often remember the Amsterdam criteria as the 3-2-1 rule. The Bethesda guidelines are a list of situations where the tumour should be tested.
The Amsterdam criteria
A family meets them only if every one of these is true.
- Three relatives with a Lynch-related cancer
- One is a parent, child or sibling of the other two
- Two generations in a row affected
- One diagnosed before the age of fifty
- A polyp syndrome called FAP ruled out
- Diagnoses confirmed by pathology reports
The Bethesda guidelines
A bowel tumour should be tested if any one of these applies.
- Bowel cancer diagnosed under fifty
- Two Lynch-related cancers in one person
- Typical Lynch features under the microscope, under sixty
- A close relative with a Lynch cancer under fifty
- Two or more relatives with Lynch cancers at any age
Which cancers count as Lynch-related
Bowel and womb cancer are the main ones. Cancers of the ovary, stomach, small bowel, urinary tract, pancreas and bile duct also count, along with certain skin and brain tumours.
Where both fall short
Small families, relatives who died without a diagnosis, and cancers never confirmed on a report all make the checklists harder to meet. In India these gaps are common, so a family can carry Lynch and still fail both.
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How does a doctor use these criteria now?
The family tree is drawn
A counsellor records who had which cancer, at roughly what age, on both sides. Approximate ages and unconfirmed diagnoses are still worth writing down.
The history is checked against the criteria
The counsellor looks at the Amsterdam and Bethesda checklists, and often a computer prediction model, to judge how likely Lynch is.
The tumour is tested if possible
A stain on the tumour sample shows whether the repair proteins are missing. This is often the quickest route and needs no extra blood.
A germline blood test follows
If the tumour or history points to Lynch, a blood or saliva test looks for an inherited fault in the repair genes. This is the test that gives the answer.
Relatives are offered testing
Once a fault is found, relatives can be tested for that exact fault, whether or not the family ever met the checklists.
On your letter
What do the terms around these criteria mean?
- Mismatch repair genes
- Genes that fix copying mistakes when cells divide. An inherited fault in one of them causes Lynch syndrome. The main ones are MLH1, MSH2, MSH6 and PMS2.
- First-degree relative
- A parent, brother, sister or child. A second-degree relative is a grandparent, uncle, aunt, niece, nephew or half-sibling.
- MMR IHC
- A stain on a tumour sample that shows whether each repair protein is present. A missing protein points towards Lynch.
- MSI
- Microsatellite instability. A tumour test showing that copying mistakes have built up, a sign that repair is not working.
- Prediction model
- A calculator such as PREMM5 that estimates the chance of Lynch from personal and family history.
- Pathology-confirmed
- A diagnosis backed by a laboratory report on tissue, rather than remembered by the family.
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Side by side
How do the two checklists compare?
Being straight with you
What this page cannot tell you
It cannot tell you whether your family has Lynch syndrome. Ticking the boxes on this page is a reason to ask for an assessment, not an answer. A genetic counsellor will look at the whole family, including relatives whose cancers were never confirmed, and decide which test makes sense.
It cannot interpret a result you already have
What your specific variant means is a question for the counsellor who ordered the test. The same applies to a tumour report that mentions MMR or MSI. A missing repair protein has more than one possible cause, and some of those causes are not inherited.
Who this does not apply to
A family with one older relative who had bowel cancer, and no other pattern, usually does not need Lynch testing. Most people do not need this test. If a relative's tumour was already checked and the repair proteins were normal, Lynch becomes much less likely, and a counsellor will explain what that means for you.
Counselling in Telugu can help older relatives describe the family history in their own words.Commonly believed
Four things families tell us, and what is actually true
Many families with Lynch syndrome do not meet them, especially smaller families. Testing the tumour, or the person with cancer, finds far more carriers than the checklist alone.
Some families meet the Amsterdam criteria and no inherited repair fault is ever found. Doctors call this familial colorectal cancer type X. Those families are still watched closely.
Womb cancer is as important, and in many women it is the first Lynch cancer to appear. Cancers of the ovary, stomach and urinary tract also count towards the pattern.
Unconfirmed cancers still help the counsellor judge the pattern, even if they do not meet the formal criteria. Write down what the family remembers, however vague it seems.
Questions we are asked
Common questions about the Amsterdam and Bethesda criteria
What is the difference between Amsterdam I and Amsterdam II?
The first version counted only bowel cancer. The second version added other Lynch-related cancers, such as womb, small bowel and urinary tract cancer. Amsterdam II is the one used today when doctors describe a family as meeting the criteria.
My father had bowel cancer at forty. Do I need testing?
A bowel cancer before fifty meets the Bethesda guidelines, so his tumour or his blood should ideally be tested first. If that is not possible, you can still be referred. A counsellor will decide which test makes sense for you.
Are these criteria used in India?
Yes, Indian oncologists and counsellors use them, but they work less well here. Families often lack pathology reports, and cancers in older relatives may never have been diagnosed. That is one reason testing the tumour itself is so useful.
What if my relative's tumour was never tested?
Stored tissue blocks from their surgery can often still be tested, even years later or after the relative has died. Ask the hospital that did the operation. If no tissue exists, a counsellor may offer a blood test to you directly.
Does a normal MMR stain rule out Lynch?
It makes Lynch much less likely for that tumour, but not impossible. If the family history is strong, a counsellor may still recommend a germline test. Ask what the stain means for your family in particular.
Do womb cancers count the same as bowel cancers?
Yes. Womb cancer is one of the main Lynch cancers and counts under both Amsterdam II and Bethesda. Many guidelines recommend checking every womb cancer for signs of Lynch, in the same way as bowel cancer.
We have a large, close-knit family. Does that change anything?
A large family makes patterns easier to see, and in communities where people marry within the family, several branches may share the same fault. It also means more relatives who can be tested once a fault is found.
Where do I start?
Write down each relative with cancer, the type, and roughly how old they were, on both sides. Take that list to a genetic counsellor or your oncologist. Call the CION helpline and we will help you arrange an assessment.
Meet CION's oncologists. Bring your family history or genetic report to them.
Our medical oncologists see people with a strong family history of cancer, arrange genetic counselling and testing where it fits, and plan the checks that follow.
Dr. C. Raghavendra Reddy
MBBS(Gold Medal), DNB(General Medicine), DM(Medical Oncology)(Gold Medal)
Dr. Bharati Devi Gorantla
MBBS, MD(General Medicine), DM(Medical Oncology)(Adyar,Chennai), ECMO, MRCP SCE(UK)
Dr. Owais Mohammed
MBBS, MD (General Medicine), DrNB (Medical Oncology), ECMO, MRCP SCE (Medical Oncology) (UK)
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Sources
- GeneReviews (NCBI) — Lynch Syndrome
- MedlinePlus Genetics — Lynch syndrome
- National Cancer Institute — Genetics of Colorectal Cancer (PDQ) – Health Professional Version
- Journal of the National Cancer Institute — Revised Bethesda Guidelines for hereditary nonpolyposis colorectal cancer (Lynch syndrome) and microsatellite instability
This page is general information, not a prescription. Do not change or stop any treatment based on what you read here. If anything is worrying you, contact your own treating team — or call our helpline and we will help you reach the right specialist.
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Does your family history fit either checklist?
Tell us who in your family had cancer, which type and at roughly what age. We will tell you honestly whether a Lynch assessment is worth arranging, and set it up if it is. One helpline serves every CION centre.