Multiple endocrine neoplasia 2 (MEN2)
Multiple endocrine neoplasia 2 (MEN2) is a rare condition where tumours develop in the endocrine system.
We hope this information answers your questions. If you have any further questions, you can ask your doctor or nurse at the hospital where you are having your treatment.
MEN stands for multiple endocrine neoplasia. It’s a rare condition caused by a faulty gene, which can be passed on within families from one generation to another (inherited).
In multiple endocrine neoplasia, a number of different tumours develop in the endocrine system. The word neoplasia means 'new growth' or 'tumour'.
There are two types of MEN: MEN1 and MEN2. Because they have similar names, MEN1 and MEN2 are often confused. In fact, they are completely separate conditions with different genetic causes. MEN1 can't turn into MEN2, and MEN2 can't turn into MEN1.
MEN2 tumours can be non-cancerous (benign) or cancerous (malignant). Malignant tumours can spread to other parts of the body, but benign tumours do not spread.
MEN2 is diagnosed when a person has more than two endocrine tumours in the body and is found to have a faulty gene (genetic mutation).
A person can also be diagnosed with MEN2 if they have one endocrine tumour and one or more members of their family have been diagnosed with MEN2.
The endocrine system is a network of endocrine glands and nerves throughout the body. Endocrine glands produce and release hormones that circulate around the body in the blood.
Hormones keep an even balance of chemicals and fluid within the body, and help us respond to changes in the environment. Normally, the hormones released by endocrine glands are carefully balanced to meet the body's needs.
The endocrine glands include the pituitary gland (which lies directly underneath the brain), the thyroid gland (in the front of the neck), the parathyroid glands (just behind the thyroid gland), and the adrenal glands (on top of the kidneys in the abdomen).
MEN2 is a very rare condition that can affect men or women at any age. Although MEN2 tends to be an inherited condition, known as familial MEN2, it can occasionally occur in people who don't have any family members diagnosed with it – this is known as sporadic MEN2.
If a person has MEN2, they are known as a MEN2 carrier. There is a 1 in 2 (50%) chance that they will pass the genetic mutation on to their children.
MEN2 occurs because of an inherited genetic change (mutation). We all inherit our genes from our parents. Genes affect the way our bodies grow, work and look.
In people with MEN2 there is a mutation, or mistake, in one gene of every cell. This mutation causes tumours to develop in the endocrine system. It isn't known exactly what causes this genetic change – research is ongoing to try to find this out.
MEN2 is divided into three subtypes, which are:
familial medullary thyroid cancer (familial MTC).
Most people with MEN2 develop a type of thyroid gland tumour called a medullary thyroid carcinoma. This is a malignant tumour.
Some people who have MEN2 develop a type of tumour of the adrenal gland called a phaeochromocytoma.
People with MEN 2A commonly develop hyperplasia (overactivity and enlargement) of the parathyroid glands.
People with MEN 2B develop tumours that appear as swellings on their tongue, lips, eyelids or lining of their mouths. These are called neuromas and are benign.
People with familial MTC tend to get medullary thyroid carcinoma only.
The symptoms for people with MEN2 can vary according to whether or not tumours develop and where these tumours are. Symptoms often occur because a tumour is making too many hormones. The particular symptoms depend on:
the type of MEN2
the type of tumour
the type of endocrine glands involved
the particular hormones that are being overproduced.
The main endocrine tumours that occur with MEN2, and their symptoms, are listed below. It's important to remember that these tumours can affect people in different ways, and that the symptoms can be similar to those of many other non-cancerous conditions.
Medullary thyroid carcinoma (cancer of the thyroid gland)
Symptoms can include:
a painless lump in the neck
difficulty breathing or swallowing due to the tumour pressing on the windpipe (trachea) or gullet (oesophagus).
Phaeochromocytoma (tumour of the adrenal gland)
These tumours are usually benign but may occur in both adrenal glands. Symptoms can include:
sudden attacks of panic and anxiety
looking pale (pallor)
looking red (flushing)
awareness of a racing heartbeat (palpitations)
high blood pressure (hypertension)
Hyperplasia (of the parathyroid glands)
Hyperplasia of the parathyroid glands can occur in people with MEN 2A. Symptoms can include:
Screening and genetic testing for MEN2
MEN2 is due to a change in a gene (mutation). We all have many thousands of genes. We have two copies of every gene, one inherited from our mother and the other inherited from our father.
People with MEN2 have one normal MEN2 gene and one MEN2 gene with an alteration (mutation). They can pass on either copy to their children, so there is a one in two (50%) chance that a child of someone with MEN2 will inherit the condition. The chance is the same for each child, regardless of their sex.
It is possible to identify the gene change in an affected person by doing a genetic test. This involves analysing the DNA (genetic code) from a blood sample. This can be a time-consuming process that often takes a number of months.
Before genetic testing is carried out, counselling is arranged to help people understand how the test results may affect them individually and as a family.
If a gene change is found, other family members can then be tested to see if they have inherited the same gene change. If they haven't, then they don't need any monitoring. However, if other relatives have inherited it, they will need to be closely monitored to detect any possible complications at an early stage.
Preventative surgery to remove the thyroid gland, before it becomes cancerous, may also be recommended.
The treatment for MEN2 depends on which tumours have developed. It can involve surgery to remove the tumours, or medicines to help control the symptoms.
Unfortunately, there is no way of changing the genetic mutation. However, problems caused by MEN2 can often be recognised at an early stage and successfully treated or controlled. Careful monitoring enables your doctor to adjust your treatment as needed, and to check for any new problems that MEN2 may cause.
Research into treatments for MEN2 is ongoing and advances are being made. Doctors use clinical trials to assess new treatments. Before any trial is allowed to take place, an ethics committee must have approved it and agreed that the trial is in the interest of the patients.
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 involves. You may decide not to take part, or to withdraw from a trial at any stage. You will still receive the best standard treatment available.
The need for practical and emotional support is different for each person. Some people with MEN2 may find that their life is largely unaffected, but for others the condition may be a source of great fear and distress.
You may have many different emotions including anger, resentment, guilt, anxiety and fear. These are all normal reactions, and are part of the process many people go through in trying to come to terms with their condition.
Everyone has their own way of coping with difficult situations. Some people find it helpful to talk to family or friends, while others prefer to seek help from people outside their situation. Some people prefer to keep their feelings to themselves.
There is no right or wrong way to cope, but help is there if you need it. You may wish to contact our cancer support specialists for information about counselling.
AMEND: The Association for Multiple Endocrine Disorders
AMEND is a service for patients, families and friends affected by MEN disorders.
This fact sheet has been compiled using information from a number of reliable sources, including:
Clark, et al. Endocrine Tumours. 2003. BC Decker Inc.
Kelsen, et al. Gastrointestinal Oncology: Principles and Practice. 2002. Philadelphia, Lippincott Williams and Wilkins.
Raghavan, et al. The Textbook of Uncommon Cancers. 3rd edition. 2006. Wiley.
Souhami, et al. Oxford Textbook of Oncology. 2nd edition. 2001. Oxford University Press.
Thanks to Professor John Monson, Professor of Clinical Endocrinology, and the people affected by cancer who reviewed this edition. Reviewing information is just one of the ways you could help when you join our Cancer Voices network.