Introduction
Being told something new that we do not quite understand can be very frightening, especially when it concerns our health and involves words like "tumour" and "cancer".
As someone newly diagnosed with a neuroendocrine tumour, or NET for short, you may be feeling scared and confused. You may have lots of questions you would like to ask your hospital doctor and general practitioner (GP), but are unsure where to begin.
By providing you with some basic information about NETs, the following information is designed to help you take your first steps on the journey towards understanding and coping with your condition. The information provided here may help you make better sense of your diagnosis and any treatments being suggested. It is written in everyday language avoiding medical terminology and it is hoped that it may also help to take away some of your fears.
The information in this section is part of a comprehensive information pack for patients which is currently being updated.
Diagnosis
What are NETs?
NETs are relatively rare, usually slow-growing tumours. In some cases, NETs are non-cancerous (benign), while in others, they are cancerous (malignant). Most NETs usually first occur in the digestive system, which is made up of the stomach and intestines, but they can arise in other parts of the body such as the lungs and pancreas and can also spread to places like the liver. When spreading occurs, doctors refer to the new NETs as "secondary tumours" or "metastases". The site where the tumour originated is known as the "primary".
Are NETs common?
No. In the UK, there are less than 2,000 new cases of NETs diagnosed each year. However, because NETs grow so slowly and in some people, produce such minor or no symptoms, doctors believe that there are probably thousands of people living their lives with NETs, but unaware of it.
Sometimes, it is only after spreading has occurred that NETs are discovered. It is not uncommon for NETs to be found unexpectedly during tests or treatments for other conditions, most commonly irritable bowel syndrome (IBS), Crohn's disease, peptic ulcer disease, gastritis or gallstones.
NETs are sometimes referred to as the 'quiet' cancer since many people with NETs experience no symptoms and are often unaware that they have a tumour.
What are the different types of NETs?
There are a number of different types of NETs. Carcinoid tumours are the most common type of NETs and may often be cancerous. They account for about 55% of all NETs diagnosed. Carcinoid tumours mainly arise in the gut and the most common sites are the appendix, the end region of the small bowel called the ileum, the stomach and the lungs.
Some NETs develop in the pancreas. These are known as pancreatic NETs and have names such as insulinomas, gastrinomas, VIPomas and nonfunctioning pancreatic NETs.
Other rarer NETs are associated with a condition called Multiple Endocrine Neoplasia type 1 or MEN-1 for short. MEN-1 is often (but not always) an inherited family condition.
What causes NETs?
Like all cancers, NETs arise when cells multiply uncontrollably in the body. Normal cells divide in a controlled manner, but in cancer, the control signals go wrong. Abnormal cells are formed, which divide continuously, resulting in the growth of a tumour. In the case of NETs, what triggers this process is unknown.
NETs occur in both men and women, although they are slightly more common in women. They can occur in adults of all ages.
Is there a link between NETs and smoking?
There appears to be no link between NETs and smoking, or NETs and any particular kind of lifestyle or occupations. However, smoking may make some treatments (like somatostatin analogues) less effective.
How will my NET affect me?
Different NETs affect people in different ways in terms of how they grow, the symptoms they produce, whether or not they spread and how they spread. However, all NETs share some characteristics.
Most NETs produce abnormally large amounts of hormones, which are chemicals made by the body to trigger its various functions. For example, most carcinoid tumours produce serotonin, an important hormone, which causes blood vessels to open and close, but which in excess can cause carcinoid syndrome, characterised by diarrhoea, stomach pain and skin flushing.
Carcinoid syndrome can also cause loss of appetite, weight loss and wheezing. Other common NET symptoms may include back pain, sickness and jaundice.
What tests will be carried out?
The tests you have and the order in which you have them will depend on your individual case.
Markers
If a neuroendocrine tumour is suspected, the doctor may want to confirm the diagnosis by measuring certain markers such as hormone levels in blood and urine samples. You may be asked to provide one or more urine samples or blood samples.
Scans
After the initial examination and blood samples, the doctors may want to use imaging equipment to look inside your body. This will confirm the tumour's position and check whether or not its tumour cells have spread to other parts of the body.
A radiolabelled octreotide scan or MIBG (Meta Iodo Benzyl Guanidine) scan may be used to show where the tumour is. A CT or CAT (Computerised (Axial) Tomography) scan can be used to give a three dimensional picture of the inside of the body and is completely painless. Some hospitals also perform MRI (Magnetic Resonance Imaging) scans. These scanners use magnetism rather than X-rays to take pictures of the inside of the body and can provide a lot of information about the position and size of a tumour, as well as showing if the tumour has spread. Some patients may not be suitable for MRI, for example those with pacemakers.
MRI scans are painless, but you have to lie very still on a couch inside the scanner. Scans can take up to an hour to complete so occasionally some people can feel claustrophobic, especially as the scanners themselves may make quite a noise.
You may also be given an ultrasound scan, which is completely painless and uses sound waves to form a picture of the inside of the stomach.
Endoscopy
Doctors may need to examine inside your digestive tract with a flexible fibre optic tube called an endoscope. The examination is called an endoscopy. You will be given medicine to help you relax before the endoscope is inserted either down the back of your throat (called a gastroscopy) or into the anus/rectum (called a colonoscopy). If the doctor sees what they think is a tumour, they may perform a biopsy.
Biopsy
You may have had a biopsy before you are referred to the specialist. A biopsy involves taking a small sample of tumour tissue. This is sent for examination under a microscope to find out whether it is benign or malignant. Depending on your other tests you may not necessarily need to have a biopsy.
Do I have to stay in hospital when I have these tests?
Not necessarily. Endoscopies are often performed on an out-patient basis. However, in some cases where a number of these tests are being performed one after the other, and particularly if you live some distance away from your nearest hospital, you may be admitted onto a ward.
Will I have to have these tests regularly?
You may have to have some of these tests more than once. Doctors may use tests like these to monitor any changes in your condition.
Treatment and care
What treatments can I have?
Your treatment depends on a number of factors. These include the type, size and position of your NET, whether it has spread from its original site to another part of your body and your general health. Treatment is aimed at both controlling the symptoms and reducing the size of the tumour.
The results of your tests will help to decide the best treatment plan for you and this will be fully explained by your doctor and discussed with you.
What sorts of medicine can I take to help my symptoms?
A commonly prescribed medicine to treat the symptoms of people with NETs is a somatostatin analogue.
Somatostatin is a naturally occurring hormone that inhibits the release of hormones and other chemicals from our internal organs. We are now able to manufacture synthetic versions (analogues) of somatostatin to use in medicines.
How do these medicines work?
These medicines work to ease symptoms and make people with NETs feel better by reducing the levels of hormones secreted by their tumours.
Do these medicines have to be injected?
Somatostatin analogues (lanreotide or octreotide) need to be injected either daily, fortnightly or monthly. The monthly formulations are becoming the most popular in terms of convenience for the patient. Many people are now treated with somatostatin analogues at their GP's surgery or at home.
Will I need an operation?
Your doctor may suggest surgery to you as a treatment option. If your NET is contained in one area (localised) or if there has been only limited spread, surgery may allow the tumour to be removed completely. Surgery can also be used to reduce the size of the tumour, which means that there is less tumour for the other treatments to take care of.
Will I need to have chemotherapy or radiotherapy?
Not all people with NETs need to have radiotherapy or chemotherapy, but they are both treatments that doctors may use. They can be used as individual treatments in their own right or as follow-up support to surgery.
Chemotherapy is the use of anti-cancer (cytotoxic) medicines to kill, slow down or stop the tumour cells dividing and multiplying. It usually involves a combination of anti-cancer medicines injected into the vein with repeated cycles of treatment.
Radiotherapy involves the use of external beam X-rays to treat localised areas of disease and bone metastases.
Will my hair fall out?
People who have cancer treatments such as chemotherapy and radiotherapy worry about the side-effects. For many, these worries focus initially on the well-documented possible side-effects such as sickness and hair loss. However, not all chemotherapy causes hair loss. The sickness may be very well controlled by taking other medicines.
If you are worried about any aspect of your treatment, tell your fears to your doctor or nurse. Your healthcare team can use the various options available to devise the most suitable treatment programme for you.
What is meant by interferon biotherapy?
Interferon is a medicine that stimulates the body's natural defence system - the immune system - and so can help the body fight NETs. It may be suitable for some patients with NETs and may often be used in combination with somatostatin analogues.
Interferon is usually self-injected. This means you can inject yourself at home either three times a week or weekly. Interferon comes in a 'pen' format that is easy to inject - your nurse will show you how to do this. Some people are not happy to do this themselves so the nurse will inject the treatment for them.
What are the different types of treatment for liver NETs?
Different techniques are used to reduce the size or slow down the growth of NETs.
Liver embolisation
Embolisation may be used to slow down the growth of secondary tumours in the liver. This involves the injection of small particles and/or a chemical into the blood vessels supplying the tumour. This cuts off the blood supply to the liver tumour and its cells begin to die. A similar procedure to this may also be performed with chemotherapy or targeted radiotherapy (see below).
Cryotherapy
Cryotherapy is used to reduce the size of tumour by freezing it with a probe.
Radiofrequency ablation
Radiofrequency ablation is used to reduce the size of the tumour by heating the tumour with radiofrequency waves through a probe.
Laser ablation
Laser ablation is used to reduce the size of a tumour by heating the tumour with a laser beam through a probe.
What are targeted radionuclide or 'magic bullet' therapies?
These are treatments that make use of the uptake of a special dye by either the octreotide scan or MIBG scan. In patients with a positive scan, the special dye is swapped for a radioactive chemical which is carried directly to the tumour by either the octreotide or MIBG.
Who will give me my treatment?
NETs are often diagnosed and treated by different hospital doctors. Your GP may have referred you to a gastroenterologist, who is a specialist in diseases of the digestive tract, surgeons who operate to try and remove the tumour, or an endocrinologist, who specialises in hormone conditions. You may then have met oncologists, who are specialist cancer doctors, and radiologists and nuclear medicine physicians, who specialise in diagnostic imaging techniques (the scans and X-rays).
It may seem that you have been passed from doctor to doctor, but what is important is that you are receiving the appropriate expert care and sometimes, it may be necessary to go through these various channels in order to get it. Whatever point you are at, your GP or nurse are there to guide and support you.
Will I be in any pain?
One in three people with cancer have no pain at all. The causes of pain and how it can be managed are well understood, so there is rarely any need for anyone to have uncontrolled pain.
People feel pain differently. Your experience of pain may be unique and would need to be treated accordingly. If you do have any pain, you should tell your doctor or nurse and they will be able to treat it.
Does the pain mean my NET is getting worse?
Having pain does not necessarily mean that the NET is getting worse. Tell your nurse or doctor about the pain, and they will be able to help you.
Can a nurse come to see me at home?
This is something that you may be able to arrange with your GP. If a somatostatin analogue has been prescribed for you, your nurse will be able to give you your injections at either your GP's surgery or in your own home. The nurse can also show you how to inject interferon at home. Additionally, it may be possible for a cancer nurse such as a Macmillan nurse to visit your home and they can help with pain relief and general care and advice.
Can I continue to lead a normal life during my treatment?
That depends on you and your type of NET. Some people diagnosed with NETs are made aware of their condition, but receive no active treatment. Instead, they are told to live their lives as before, but are monitored through regular screening (e.g. with the tests described previously) to check for any changes in their condition.
This is because NETs are usually slow-growing and in some people do not produce symptoms that affect their day-to-day lives.
For other people with NETs, life may be disrupted by having to go into hospital for surgery or other treatment. This may mean that you will have to get used to taking medicines such as somatostatin analogues regularly. The aim is that your treatment controls your symptoms to hopefully improve your quality of life. There are some things you can do to help yourself and this is dealt with in the Self Help and Support section.
What is palliative care?
Palliative care is an important aspect of your care. It focuses on the quality of your life and not only assists in controlling any pain that you may have, but it also provides help and support for social, emotional and spiritual needs.
Palliative care is available for all cancer patients.
Where do I get palliative care from?
This care is provided by a team of specialist health professionals working from your hospital or in the community. You can either discuss palliative care with your doctor or nurse, or you will find that the organisations listed in the Further Help and Support section provide information, support and advice about this aspect of care.
Self-help and support
How can I help myself?
There are a number of ways that you can help yourself. It is very easy to feel helpless, alone and depressed when diagnosed with cancer. But many people with NETs and other forms of cancer will tell you that their condition has actually had a positive effect on their life. They have stopped worrying about the more inconsequential niggles of day-to-day living and started to do more things that they enjoy such as hobbies and spending time with friends and loved ones. Many people find it empowering to learn about their condition. You may benefit by joining support groups and talking to others in the same or similar situation to yourself. Support groups can often also provide advice on entitlements and benefits that may be available to you.
Do I have to change what I eat?
Some people use their diagnosis as a spring-board to a healthier lifestyle. They may stop smoking, cut down on alcohol and make a conscious effort to eat more fruit and vegetables and less fatty foods. Vitamin supplements may also be of benefit. Following a healthy diet and if possible, taking regular, gentle exercise may help you feel better, not least because you are doing something positive. Taking a positive attitude may also contribute to the effectiveness of your treatment. Your medical team may give specific recommendations about your diet.
What should I do if I can't work?
If you are unable to work as a result of having a NET then this does not necessarily mean that your life will be affected negatively. Many people find happiness and interest in other activities and may even do something they have not done before. For information on welfare and benefits that you may be entitled to, contact CancerBACUP (see below).
Further help and support
CancerBACUP
3 Bath Place
Rivington Street
London EC2A 3JR
Tel: 0808 800 1234 Fax: 020 7696 9002
Website: www.cancerbacup.org.uk
NET Patient Foundation (incorporating Living with Carcinoid)
Website: www.netpatientfoundation.com
Macmillan Cancer Relief
89 Albert Embankment
London SE1 7UQ
Helpline: 0808 808 2020
Tel: 020 7840 7840 Fax: 020 7840 7841
Website: www.macmillan.org.uk
NHS Direct
Tel: 0845 4647
Website: www.nhsdirect.nhs.uk
Tak Tent Cancer Support Scotland
Flat 5, 30 Shelley Court
Gartnaval, Glasgow G12 0YN
Helpline: 0141 211 0122 Fax: 0141 211 0010
Website: www.taktent.org
Tenovus
9th Floor, Gleider House, Ty Glas Road
Llanishen, Cardiff, CF14 5BD
Helpline: 0808 808 1010
Tel: 029 2076 8850 Fax: 029 2076 8888
Website: www.tenovus.com
The Ulster Cancer Foundation
40-44 Eglantine Avenue
Belfast BT9 6DX
Helpline: 0800 783 3339
Website: www.ulstercancer.org
The Carcinoid Cancer Foundation (USA)
333 Mamaroneck Avenue # 492
White Plains
NY 10605 USA
Helpline:+ 1 888 722 3132 or + 1 914 683 1001
Website: www.carcinoid.org
