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An overview of endocrine tumours

The endocrine system is a network of endocrine glands in 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.

The endocrine system is made up of several glands, including: the pituitary gland, thyroid gland, the pancreas, parathyroid glands and the adrenal glands.

Tumours can occur in endocrine glands. Most endocrine tumours are non-cancerous (benign), but some are cancerous (malignant).

Endocrine tumours are classified according to:

  • the particular endocrine glands that are involved
  • the type of cell from which the cancer developed
  • the part of the body that is affected

Endocrine tumour:

  • Adrenal gland tumours
  • Multiple endocrine neoplasia 1 (MEN1)
  • Multiple endocrine neoplasia 2 (MEN2)
  • Pancreatic cancer
  • Parathyroid gland tumours
  • Pituitary gland tumours
  • Thyroid cancer

The endocrine system

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. The endocrine system is made up of several glands, including the thyroid gland, the parathyroid glands, and the pituitary gland.

Endocrine tumours

Endocrine tumours are a rare group of tumours that start in the cells of the endocrine system. Most endocrine tumours are non-cancerous (benign), but some are cancerous (malignant).

Endocrine tumours are classified according to:

  • the particular endocrine glands that are involved
  • the type of cell from which the cancer developed
  • the part of the body that is affected.

The adrenal glands

There are two adrenal glands. One sits on the top of each kidney. The adrenal glands produce a number of vital hormones essential for survival.

There are two parts of the adrenal glands. The inner part is known as the medulla, and the outer part of the gland is called the cortex.


Position of the adrenal glands
Position of the adrenal glands

When a tumour develops in the adrenal glands, it often causes too much of a particular hormone to be produced. The type of hormone overproduced depends on the part of the adrenal gland that is affected by the tumour.

Some endocrine tumours may not cause an overproduction of hormones and do not cause any obvious symptoms. These are known as non-functioning tumours.


The adrenal cortex

The adrenal cortex produces different types of hormones. These are known collectively as steroids. They regulate different mechanisms within the body. The different types of steroids are:

  • Glucocorticoids regulate the way that the body uses carbohydrate, protein, and fat. They also affect the levels of glucose in the blood.
  • Mineralocorticoids control the balance of sodium (salt) and water in the body, which maintains the amount of blood in the heart and circulatory system, and regulates blood pressure.
  • Sex hormones Although the male hormones (androgens) and female hormones (oestrogen and progesterone) are mainly produced by the testes and ovaries respectively, the adrenal cortex also produces small amounts of androgens in both men and women.

The adrenal medulla

The adrenal medulla produces the hormones adrenaline (epinephrine) and nor-adrenaline (norepinephrine). These hormones make the heart beat faster and cause sweating, an increased blood supply to vital organs, slowing of digestion and dilatation of the pupils of the eye. These effects are important in helping the body to react to emergency situations. Adrenaline and nor-adrenaline are sometimes called the hormones of 'fight or flight'.

Types of adrenal tumours

Tumours of the adrenal gland can develop in either the cortex or the medulla.

Benign tumours of the cortex are called adrenal cortical adenomas. Malignant tumours are called adrenal cortical carcinomas.

The most common type of tumour in the medulla is called a phaeochromocytoma (pronounced fee-oh-cromo-sigh-toma). A small number of phaeochromocytomas start outside the medulla part of the adrenal gland and are known as extra‑adrenal phaeochromocytomas.

Usually, only one adrenal gland is affected. Rarely, tumours may occur in both adrenal glands; these are known as bilateral adrenal tumours.

Causes of adrenal cancer

As with many other forms of cancer, the exact causes of adrenal cancer are usually unknown. However, the risk of developing an adrenal tumour is increased in people who have Multiple Endocrine Neoplasia (MEN1 and 2).

MEN1 and MEN2 are very rare conditions caused by an inherited faulty gene. MEN1 is associated with adrenal adenomas, and MEN2 is associated with phaeochromocytoma (which can be malignant). People who have relatives with MEN can ask their GP to refer them to a family cancer clinic for advice and for genetic testing.

Signs and symptoms

Functioning tumours may cause symptoms because they produce excess amounts of particular hormones. The symptoms will depend on the hormone that is being overproduced.

When a tumour does not produce high levels of hormones (is non-functioning), it can start to cause symptoms after a period of time because of its position or size. Pressure on surrounding organs may cause symptoms such as pain.

Carcinoma of the adrenal cortex

About 2 out of 3 adrenal cortical carcinomas cause symptoms by producing high levels of one or other of the adrenal cortex hormones. The particular hormone being overproduced will determine the symptoms, with some tumours producing more than one class of steroid.

Glucocorticoid This can lead to weight gain, particularly in the trunk area, while there is usually some muscle wasting of the limbs, making them appear thinner. There can also be an accumulation of fat at the base of the neck, which can lead to a 'buffalo hump' appearance. There can also be a swelling of the cheeks and face, giving a 'moon face' appearance.

Some people have mild diabetes, which can cause an increase in thirst and a need to pass urine more frequently. Other symptoms include high blood pressure (hypertension), weakened bones (osteoporosis), depression, and abnormal hair growth; fine, downy hair grows on the face, arms, and upper back. These symptoms, combined, are often called Cushing's syndrome.

Mineralocorticoids This can lead to an increase in blood pressure, muscle cramps, thirst, and excessive passing of urine. However, there are many other causes of raised blood pressure which are much more common than adrenal cortical cancers.

Sex hormones In women, this can lead to the development of male features such as a deepening of the voice and development of baldness. Periods may also be disturbed. In men, excess production of sex hormones may cause a loss of libido, impotence, and sometimes some swelling and enlargement of the breasts.

Tumours of the adrenal medulla

Phaeochromocytoma is the most common type of adrenal medulla tumour. Symptoms include:

  • sudden attacks of panic and anxiety
  • headaches
  • pallor or flushing
  • sweating
  • palpitations (a racing heartbeat)
  • hypertension (high blood pressure)
  • irritability and mood changes
  • weight loss.

Most phaeochromocytomas are benign, but they can be malignant. If you notice any of the above symptoms, contact your GP, but remember that these symptoms can also be caused by other conditions.

Diagnosis and tests

Tumours of the adrenal gland can be very difficult to diagnose. Usually, you begin by seeing your GP who will examine you and refer you to a hospital specialist.

The hospital specialist will organise any tests that may be necessary, and can give you expert advice and treatment. The doctor will take your full medical history, carry out a physical examination, and take blood samples to check your general health. The following tests may be carried out:

Blood and urine tests Several samples of blood and urine are taken to look for the levels of certain hormones. Samples of urine may be collected over a 24 hour period. Sometimes it is necessary to fast overnight before having certain blood tests taken. These tests may be repeated to monitor any consistent changes in hormone levels.

X-rays and scans A combination of x-rays and scans will be taken to locate the tumour and find out if it has spread.

Abdominal ultrasound Sound waves are used to make up a picture of the abdomen and surrounding organs. It is done in the hospital scanning department. You will be asked not to eat, and to drink clear fluids only (nothing fizzy or milky) for 4–6 hours before the scan. Once you are lying comfortably on your back, a gel is spread onto your abdomen. A small device, like a microphone, is then rubbed over the area. The sound waves are converted into a picture using a computer. The test is completely painless and takes about 15-20 minutes.

CT (computerised tomography) scan A CT scan takes a series of x-rays to build up a three-dimensional picture of the inside of the body. The scan is painless but takes about 30 minutes. It may be used to find where the cancer started (primary tumour) or to check for any spread of the disease (metastases).

MRI (magnetic resonance imaging) scan This scan uses magnetism instead of x-rays to form a series of cross-sectional pictures of the inside of the body. During the scan, you will be asked to lie very still on the couch inside a metal cylinder. The test can take up to an hour and is completely painless. If you don't like enclosed spaces, you may find the machine claustrophobic. The machine is also quite noisy, but you will be given earplugs or headphones to wear.

123MIBG scan A drug called 123MIBG (Meta-iodobenzylguanidine) may be used to show up the site of a phaeochromocytoma. 123MIBG is mildly radioactive iodine that is taken up by adrenal cells. The scan takes place over two consecutive days in the nuclear medicine department. On the first day, you will go to the department twice. In the morning you will be given an injection in the arm. You are then free to stay in, or leave, the hospital. You will go back to the department in the afternoon to have a scan with a gamma camera, lasting for about an hour. The following morning you will have further pictures taken for about one and a quarter hours. At this point you may be given a further injection.

Biopsy A small sample of cells is taken from the tumour to be examined under a microscope. The biopsy may be carried out under a local or general anaesthetic.

Angiography An angiogram is a technique used to assess the flow of blood through the blood vessels of the adrenal glands. An injection is given into a vein in the arm, and a scan of the adrenal glands is then carried out.

Treatment

Treatment depends on a number of factors, such as the type and size of the tumour, and whether or not it has spread, and will therefore vary from person to person.

Surgery

If the tumour is contained in one area, or if there has been only limited spread, surgery is usually the first choice of treatment. If it is possible to remove the tumour completely, no other treatment may be necessary.

The type of operation depends on the size and exact position of the cancer. Sometimes, the affected adrenal gland and kidney may need to be removed.

If the tumour has spread to other parts of the body, surgery may still be possible to remove the part of the tumour that is producing too many hormones. This is often referred to as tumour debulking.

If the tumour is causing a blockage, for example, in the bowel, surgery may be helpful to relieve the blockage.

Non-surgical treatments

  • Drugs to lower blood pressure These are known as anti-hypertensive medicines.
  • Steroids Sometimes, steroids may be given as tablets or by injection into a vein to reduce the size of an adrenal tumour, or to help to control symptoms that the tumour may be causing.
  • Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. Chemotherapy may sometimes be used to treat adrenal tumours that have spread. It aims to shrink the tumour and reduce the amount of hormones it is producing.
  • Radiotherapy uses high-energy rays to destroy cancer cells. Sometimes it will be given following an operation. The aim is to destroy any remaining cancer cells. Radiotherapy may also be given to treat symptoms, such as pain.
  • Radionuclide therapy Some types of tumour may take up large amounts of particular chemicals, for example, MIBG. To treat these tumours, the chemical can be attached to a dose of a radioactive substance. As the cancer takes up the chemical, it also takes in the radioactivity. To test whether or not the tumour takes up large quantities of these chemicals, a scan will first be done using a tiny amount of radioactivity. If the tumour takes up the radioactivity, the treatment will be given using a higher dose of the radioactive substance. To have this treatment, you may need to be looked after in a room by yourself for a few days, so that other people are not exposed unnecessarily to the radioactivity.

Clinical trials

Research into treatments for adrenal tumours is ongoing and advances are being made. Cancer doctors 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 involves. You may decide not to take part, or to withdraw from a trial, at any stage. You will then receive the best standard treatment available.

Your feelings

Having a cancer diagnosis can be extremely frightening. You may experience many different emotions, including anger, resentment, guilt, 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.

Each individual has their own way of coping with difficult situations; some people find it helpful to talk to friends or family, 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 service for information about counselling in your area.

Multiple Endocrine Neoplasia 1 (MEN1)


  • The MEN types
  • The endocrine system
  • Who MEN1 affects
  • Causes
  • MEN1
  • Signs and symptoms
  • Screening and genetic testing
  • Treatment
  • Clinical trials
  • Your feelings

The MEN types

MEN stands for multiple endocrine neoplasia. It is a rare condition that is caused by a faulty gene; this can be passed on, within families, from one generation to another (inherited). There are two types: 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 cannot turn into MEN2 and MEN2 cannot turn into MEN1.

In multiple endocrine neoplasia, a number of different tumours develop in the endocrine system. The endocrine system is a network of glands in the body. The word neoplasia means new growth or tumour.

MEN1 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.

MEN1 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 as having MEN1 if they have one endocrine tumour and one or more members of their family have been diagnosed with MEN1.

The endocrine system

The endocrine system is made up of endocrine glands. Endocrine glands produce hormones; these chemicals control many of our body's functions. 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 immediately underneath the brain), the thyroid gland (in the front of the neck), the parathyroid glands (that lie just behind the thyroid gland), and the adrenal glands (which sit on top of the kidneys in the abdomen).

Who MEN1 affects

MEN1 is a very rare condition that can affect men or women at any age. Although it tends to be an inherited condition, known as familial MEN1, it can sometimes occur in people who do not have any family members diagnosed with it and it is then known as sporadic MEN1.

If a person has MEN1, they are known as a MEN1 carrier. There is a 1 in 2 chance that they will pass the genetic mutation on to their children.

Causes

MEN1 occurs because of an inherited genetic change (mutation). We all inherit many genes from our parents. Genes affect the way your body grows, works, and looks. In people with MEN1 there is a mutation, or mistake, in one gene of every cell. This mutation causes tumours to develop in the endocrine system. It is not known exactly what causes this genetic change, and research is ongoing to try to find out.

MEN1

Almost all people with MEN1 will develop overactivity of the parathyroid glands, known as hyperparathyroidism, at some stage in their life. This is often shown by an abnormally high level of calcium in the blood (hypercalcaemia). There may be tumours in the parathyroid glands, which are usually benign.

Tumours can also develop in the digestive system (gastrointestinal tract or GI tract), usually the stomach, intestines and/or pancreas. These tumours can be benign or malignant. Other tumours can develop in the pituitary gland or the adrenal glands, these are usually benign.

Rarely, people may develop a particular type of tumour (known as a carcinoid tumour) in the chest, thymus gland (behind the breast bone), or the gastrointestinal tract.

Signs and symptoms

The symptoms for people with MEN1 can vary according to whether or not tumours develop and where these tumours are. Symptoms often occur because a tumour is causing too many hormones to be produced. The particular symptoms depend on:

  • the type of tumour
  • the type of endocrine glands that are involved
  • the particular hormones that are being overproduced.

The main endocrine tumours, and some of the most common symptoms they cause, are listed below. It is 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.

Tumours of the parathyroid gland Possible symptoms include: high levels of calcium in the blood (hypercalcaemia); a possible lump or swelling in the neck; muscle weakness; pain in the bones; vomiting; loss of appetite; weight loss; constipation; difficulty sleeping; lethargy; nervousness; and stomach ulcers.

Tumours of the pituitary gland Symptoms can include headaches and sight problems. Infertility may occur. Some people have a condition called acromegaly which affects growth, leading to an enlargement of the hands and feet, and can lead to high blood pressure and diabetes. Women may notice that their periods stop, and they may produce small amounts of breast milk. Men may be unable to have an erection (impotence).

GEP tumours

There are several different types of GEP tumours (gastroenteropancreatic tumours). They tend to develop in the organs of the digestive system, such as the stomach (gastro), intestines (entero) and the pancreas.

Insulinomas Symptoms include: headaches, confusion, trembling, palpitations, anxiety, visual disturbances and possible fits (seizures).

Gastrinomas Possible symptoms include: bleeding in the stomach, black tar-like stools, or pale, greasy and offensive-smelling stools (steatorrhoea) or diarrhoea, possible narrowing of the bowel, which may cause general abdominal pain, and inflammation of the gullet lining (oesophagitis).

Somatostatinomas Symptoms can include pale, greasy and offensive-smelling stools (steatorrhoea), weight loss, anaemia, pain, diarrhoea, and possible diabetes.

Glucagonomas Symptoms such as anaemia, weight loss, diabetes, rash, or formation of blood clots can occur.

VIPomas Excessive watery diarrhoea, low levels of potassium (as a result of excessive diarrhoea), weakness, sickness (nausea), and vomiting are possible symptoms.

Tumours of the adrenal gland These tend to be 'non-functioning' which means there is not an excess production of hormones and there are no obvious symptoms.

Screening and genetic testing

MEN1 is due to an alteration in a gene (also called a mutation). We all have many thousands of genes and we have two copies of every gene, one inherited from our mother and the other inherited from our father. People with MEN1 have an alteration in one of the copies in their MEN1 gene, and one normal copy of the gene. They can pass on either copy to their children so there is half a chance that a child of someone with MEN1 will inherit the condition. The chance is the same for each child, regardless of the sex of the child.

It is possible to identify the gene change in an affected person by doing a genetic test. This involves analysis of the DNA (genetic code) from a blood sample. This can be a time-consuming process which often takes a number of months. In some cases, the gene change cannot be found although the person is affected with MEN1. This is probably due to technical limitations of the test. Before genetic testing is carried out, counselling is arranged to help people to 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 have not inherited the gene change, then they do not need any monitoring. However, if they have inherited it, then they will need to be closely monitored to detect any possible complications at an early stage.

Treatment

The treatment for MEN1 depends on which tumours have developed, and can involve surgery to remove the tumours or medicines to help control the symptoms. Although there is no way of changing the genetic mutation, problems caused by MEN1 can often be recognised at an early stage and successfully controlled or treated. Careful monitoring enables your doctor to adjust your treatment as needed and to check for any new problems that the MEN1 may cause.

Clinical trials

Research into treatment for MEN1 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 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 then receive the best standard treatment available.

Your feelings

The need for practical and emotional support is, of course, different for each person. Some people with MEN1 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 and its treatment. You don't have to cope with these feelings on your own; people are available to help you. Some hospitals have their own emotional support services with specially trained staff and some of the nurses will have received training in counselling. Cancerbackup can also put you in contact with counselling services in your area.

Multiple Endocrine Neoplasia 2 (MEN2)


  • The MEN types
  • The endocrine system
  • Who MEN2 affects
  • Causes
  • MEN2
  • Signs and symptoms
  • Screening and genetic testing
  • Treatment
  • Clinical trials
  • Your feelings

The MEN types

MEN stands for multiple endocrine neoplasia. It is a rare condition that is caused by a faulty gene; this can be passed on, within families, from one generation to another (inherited). There are two types: 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 cannot turn into MEN2, nor can MEN2 turn into MEN1.

In multiple endocrine neoplasia, a number of different tumours develop in the endocrine system. The endocrine system is a network of glands in the body. The word neoplasia means 'new growth' or 'tumour'.

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 as having MEN2 if they have one endocrine tumour and one or more members of their family have been diagnosed with MEN2.

The endocrine system

The endocrine system is made up of endocrine glands. Endocrine glands produce hormones; these chemicals control many of the body's functions. Normally, the hormones released by endocrine glands are carefully balanced to meet the body's needs.

The endocrine glands include the thyroid gland (in the front of the neck), the parathyroid glands (that lie just behind the thyroid gland), and the adrenal glands (which sit on top of the kidneys in the abdomen).

Who MEN2 affects

MEN2 is a very rare condition, which 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 do not have any family members diagnosed with it and it is then known as sporadic MEN2.

If a person has MEN2, they are known as an MEN2 carrier. There is a 1 in 2 chance that they will pass the genetic mutation on to their children.

Causes

MEN2 occurs because of an inherited genetic change (mutation). We all inherit many genes from our parents. Genes affect the way your body grows, works, and looks. 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 is not known exactly what causes this genetic change, and research is ongoing to try to find out.

MEN2

Many people with MEN2 develop a type of thyroid gland tumour called a medullary cell carcinoma. This is a malignant tumour.

Some people who have MEN2 will develop a type of tumour of their adrenal gland called a phaeochromocytoma (see the section Tumours of the adrenal gland).

Some people who have MEN2 will develop a type of tumour of their adrenal gland called a phaeochromocytoma.

MEN2 is further divided into three subtypes:

  • MEN 2A
  • MEN 2B
  • familial medullary thyroid cancer (familial MTC).

People with MEN 2A people 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. Tumours of the parathyroid glands do not occur in MEN 2B.

In familial MTC, people tend to get medullary thyroid cancer only.

Signs and symptoms

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 are listed below. It is 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.

Hyperplasia of the parathyroid glands Possible symptoms include high levels of calcium in the blood (hypercalcaemia); a possible lump or swelling in the neck; muscle weakness; pain in the bones; vomiting; loss of appetite; weight loss; constipation; difficulty sleeping; lethargy; nervousness; and stomach ulcers.

Cancer of the thyroid gland You may notice a painless lump in the neck, and possible difficulty in breathing or swallowing due to the tumour pressing on the gullet or windpipe.

Tumours of the adrenal gland Some people with MEN2 may develop a type of tumour of their adrenal gland called a phaeochromocytoma. These tumours are almost always benign but may occur in both adrenal glands. Symptoms include: sudden attacks of panic and anxiety; headaches; pallor or flushing (looking red); sweating; a racing heartbeat (palpitations); high blood pressure (hypertension); weight loss; irritability; and mood changes.

Screening and genetic testing

MEN2 is due to a change in a gene (mutation). We all have many thousands of genes, and we have two copies of every gene, one inherited from our mother and the other inherited from our father. People with MEN2 have a change in one of the copies in their MEN2 gene, and one normal copy of the gene. They can pass on either copy to their children, so there is half a chance that a child of someone with MEN2 will inherit the condition. The chance is the same for each child, regardless of the sex of the child.

It is possible to identify the gene change in an affected person by doing a genetic test. This involves analysis of the DNA (genetic code) from a blood sample. This can be a time-consuming process which often takes a number of months. Before genetic testing is carried out, counselling is arranged to help people to 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 have not inherited the gene change, then they do not need any monitoring. However, if they have inherited it, then 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.

Treatment

The treatment for MEN2 depends on which tumours have developed, and 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 and controlled, or treated, at an early stage. Careful monitoring enables your doctor to adjust your treatment as needed, and to check for any new problems that MEN2 may cause.

Clinical trials

Research into treatments for MEN2 tumours 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 then receive the best standard treatment available.

Your feelings

The need for practical and emotional support is, of course, 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 and its treatment. You don't have to cope with these feelings on your own; people are available to help you. Some hospitals have their own emotional support services with specially trained staff and some of the nurses will have received training in counselling. Cancerbackup can also put you in contact with counselling services in your area.

Tumours of the parathyroid glands


  • The endocrine system
  • The parathyroid glands
  • Parathyroid tumours
  • Causes of parathyroid gland cancer
  • Signs and symptoms
  • Diagnosis
  • Treatment
  • Clinical trials
  • Your feelings

The endocrine system

The thyroid gland and the parathyroid glands are part of the endocrine system. Although they are close to each other and have similar names, they have very different functions.

The parathyroid glands

There are four parathyroid glands, which are attached to the thyroid gland, in the front of the neck.

The parathyroid glands are small, but their function is very important: they maintain the correct levels of calcium in the body. Calcium plays an essential role in controlling muscle and nerve function.


Position of the parathyroid glands
Position of the parathyroid glands

Parathyroid tumours

Parathyroid tumours can be non-cancerous (benign) or cancerous (malignant). Most tumours of the parathyroid gland are benign. This information discusses the malignant endocrine tumours of the parathyroid glands. Cancer of the parathyroid gland is extremely rare.

A tumour of the parathyroid gland may cause the overproduction of the hormone that controls the level of calcium in the body. The hormone is called parathyroid hormone (PTH), or parathormone.

Causes of parathyroid gland cancer

The exact cause of parathyroid tumours is not fully understood, but we do know that the risk of developing it increases in people affected by a condition known as multiple endocrine neoplasia 1 (MEN1). This is a rare condition that is caused by a faulty gene; it can be passed on from one generation to another (inherited).

Cancer of the parathyroid glands has also been linked to exposure to radiation.

Signs and symptoms

Some people with a parathyroid tumour may feel a lump or swelling in their neck, but this is not common.

If parathyroid hormone is being overproduced, it may cause some of the following symptoms:

  • high levels of calcium in the blood (hypercalcaemia)
  • muscle weakness
  • pain in the bones
  • vomiting
  • loss of appetite/weight loss
  • constipation
  • difficulty sleeping
  • feeling run down and tired
  • inflammation of the pancreas
  • stomach ulcer
  • kidney stones
  • feeling nervous
  • thin bones (osteoporosis).

Some people may only have one or two of these symptoms. These symptoms may also be caused by other conditions not related to parathyroid tumours.

Diagnosis

Before a diagnosis can be made, your doctor will ask you about your past medical conditions, including any symptoms that you have.

A number of tests will be done. These may include any of the following:

Blood and urine tests Samples of blood and urine are taken to look for the presence of certain hormones. A sample of urine may be collected over a 24 hour period. Sometimes it is necessary to fast (not eat) overnight before having certain blood tests.

Parathyroid scan This scan can identify the size and position of the parathyroid glands, and how well they are working. A slightly radioactive substance is injected into a vein in your arm. The scan shows how much of the substance is taken up by the parathyroid glands, and identifies any abnormal areas.

X-rays and scans A combination of x-rays and scans will be taken to find where the tumour is, and if it has spread.

Ultrasound scan Sound waves are used to make up a picture of the neck area. Once you are lying comfortably on your back, a gel is spread over your neck. A small device like a microphone, which produces sound waves, is then passed over the area.

CT (computerised tomography) scan This is a series of x-rays, which builds up a three‑dimensional picture of the inside of the body. The scan is painless. It takes about 30 minutes.

MRI (magnetic resonance imaging) scan This uses magnetism instead of x-rays to form a series of cross-sectional pictures of the inside of the body. During the scan you will be asked to lie very still on a couch inside a metal cylinder. The test can take up to an hour and is completely painless. If you don't like enclosed spaces, you may find the machine claustrophobic. The machine is also quite noisy, but you will be given earplugs or headphones to wear.

PET (Positron emission tomography) scan This is a new type of scan and is occasionally used for this type of tumour. A PET scan uses low-dose radioactive sugar to measure the activity of cells in different parts of the body. Areas of tumour are usually more active than surrounding tissue, and show up on the scan. If you need a PET scan, you might have to travel to a specialist centre to get one.

Biopsy A small sample of cells is taken from the tumour to be examined under a microscope. A local or general anaesthetic may be given.

Treatment

Surgery

If the tumour is contained in one area (localised), or if there has been only limited spread, surgery is usually the first choice of treatment. If it is possible to remove the tumour completely, no other treatment may be necessary.

Surgery usually involves removing all of the parathyroid gland and part, or all, of the nearest half of the thyroid gland, and some surrounding tissue. After the operation, the calcium levels in the body can go up and down. Your doctors will monitor your calcium level by taking regular blood tests.

Usually, this type of surgery is done under a general anaesthetic and requires a short stay in hospital. Sometimes it is possible to remove the affected parathyroid gland using keyhole surgery, under a local anaesthetic. However, the effectiveness of this technique is still being investigated in research trials.

Radiotherapy

This is the use of high-energy rays. Radiotherapy can be given after surgery, with the aim of reducing the risk of the cancer coming back. It may also be given If your cancer comes back some time after the initial surgery, or if it has spread to another part of the body.

Chemotherapy

This uses anti-cancer (cytotoxic) drugs to destroy cancer cells. Chemotherapy is occasionally used for parathyroid cancer that has come back after initial treatment, or has spread to other parts of the body.

Bisphosphonates

Medicines known as bisphosphonates can be given to reduce high levels of calcium in the blood (hypercalcaemia).

Calcimimetic agents

These are medicines that are used to control some of the symptoms associated with an overproduction of parathyroid hormone (PTH) and hypercalcaemia.

Clinical trials

Research into treatments for parathyroid cancer is ongoing and advances are being made. Cancer doctors use clinical trials to assess new treatments. Before any trial is allowed to take place it must have been approved by an ethics committee, which will have checked whether the trial is in the interest of 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 then receive the best standard treatment available.

Your feelings

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 illness.

Each individual has their own way of coping with difficult situations; some people find it helpful to talk to friends or family, 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 service for information about counselling in your area.