Print

What are Neuroendocrine tumours?

How does it feel to be diagnosed with a Neuroendocrine tumour? Fearful, concerned and perhaps a bit confused?

One person, recently diagnosed with a NET stated: "Suddenly I found myself facing a whole collection of scans and blood tests, many of which I had never heard of. It was so frightening and I had nothing to read that seemed to relate to what I was experiencing".

Neuroendocrine tumours or NETs, is the umbrella term for a group of relatively uncommon cancers, often called the 'quiet cancers'. Around 3,000 new cases are diagnosed every year in the UK, but it is thought that a larger number of people have a NET, but remain undiagnosed. This is because NETs can be difficult to diagnose as the symptoms may be vague and attributed to more common problems such as irritable bowel syndrome (IBS), Crohn's disease, peptic ulcer disease, gastritis, asthma, blood glucose problems, menopause and blood pressure problems.

Neuroendocrine cancer is formed in the diffuse neuroendocrine system, which is made up of neuroendocrine cells found in the respiratory and digestive tracts. The respiratory tract includes the bronchial tubes and lungs. The digestive tract starts at the mouth and ends at the rectum.

Neuroendocrine cells are also found in the endocrine glands, such as the adrenal glands, pancreas, thyroid and pituitary. These cells are also found in the ovaries and the testes.

How NETs are formed is still not fully understood. As with all forms of cancer, NETs arise when cells multiply rapidly in the body. Normal cells in our body divide in a controlled manner, but in cancer the control signals go wrong. This causes abnormal cells to form, which divide quickly resulting in tumour growth.

Sites where NETs tumours may appear

NETs can be found in several organs of the body but their most common locations are in the lungs and in the digestive system. They were first identified as a specific type of disease in the mid-1800s, and the name 'carcinoid' was given to them in 1907 to describe a tumour that grew much more slowly than usual cancers. However, by the 1950s it became quite clear that these slow-growing tumours could be malignant and spread from one part of the body to another like other forms of cancer.

The terminology for NETs can be confusing but the most accurate term is simply NET with the site of the tumour specified, eg small intestinal NET (see below for other examples). Some NETs are referrred to as GEP NETs. This is a term for those tumours which arise in the cells of the stomach (gastro), intestines (entero) and the pancreas.

Most NETs occur without any genetic link, but there are cases where they are present as part of a familial endocrine cancer syndrome such as MEN1, MEN 2, neurofibromatosis type 1 or Von Hippel Lindau.

Types of Neuroendocrine Tumours

There are a number of different types of NET. They all have a slightly different way of presenting themselves, both in terms of symptoms and how they look under a microscope.

Examples of NETs

Gastrointestinal NETs: gastric, duodenal, pancreatic, small intestine, appendiceal, colon, rectal, ovarian and carcinoid tumours of unknown origin (unknown primary)
Lung NETs
Thymic NETs: Medullary Thyroid Cancer
Pancreatic NETs (non-functioning)
Pancreatic NETs (functioning): Gastrinoma, VIPoma, Insulinoma, Somatostatinoma, Glucagonoma
MEN
Phaeochromocytoma and Paraganglioma
Goblet Cell NET

The place where the NET first appears is called the 'primary' site. However, the NET may spread and be found in other parts of the body e.g. the liver, and if this occurs your doctor may refer to these as a 'secondary' tumour or metastases.

NETs are still mainly classified according to the area in which they are found:

Classification of NETs

Foregut tumours: found in the lungs, stomach, pancreas, gall bladder and duodenum
Midgut tumours: found in the jejunum, ileum, appendix and right colon
Hindgut tumours: found in the left colon and rectum

Some NETs produce abnormally large amounts of hormones. These NETs have a related syndrome which means that the hormones secreted cause noticeable symptoms like flushing, diarrhoea, cramps, wheezing, heart valve damage and skin changes.

The group of tumours that arise in the pancreas can be classified into two different groups; functioning and non-functioning. The functioning group will produce a number of clinical syndromes that are related to where they originate, for example, an insulinoma will over-secrete insulin and gastrinomas are gastrin-secreting tumours. The non-functioning group may secrete certain hormones and peptides like other NETs, but the release of these chemicals does not cause an identifiable 'syndrome' or collection of symptoms. This can make diagnosis difficult and explains why so many cases are picked up incidentally.

Different NETs Affect People Differently

Different NETs affect people in different ways in terms of how the tumour grows, the symptoms produced, whether or not they spread and how they spread.

Gaining the histology (what the tumours look like under a microscope) is very important in order to classify the cancer into a type, and the healthcare team can then work with you to plan the most appropriate treatment.

Although NETs share similar characteristics, the diagnosis and the way your cancer may behave may be different.

The most important aspect of caring for a person with a NET is that the care should be tailored to suit the individual and provided by a specialist in the field of NETs. Your quality of life is paramount and so team work is essential to provide a solid plan of treatment and follow-up.

There has been much research work done by specialist healthcare professionals, and progress has been made in terms of understanding these tumours. It is important to ensure that you are seen by these specialists in order to access all the knowledge available.

NET cancer care can be complex. For you the journey can encompass not only a whole host of emotions, but also a range of investigations, treatments and healthcare professionals. Often there is more than one treatment option available, and so there has to be a collaboration amongst all key healthcare professional groups who are making clinical decisions for you. This collaboration is called a multidisciplinary team or MDT. The MDT management approach is now being used across the world in the care of people with NETs.

Throughout the NET journey, you may potentially see the following healthcare professionals:

Healthcare professionals who may be involved in your care

  • Gastroenterologist
  • Oncologist
  • Surgeon
  • Endocrinologist
  • Radiologist
  • Nuclear Medicine Physician
  • Nurse Specialist
  • Dietitian
  • Palliative Care Team
  • Pain Team
  • General Practitioner/Practice Nurse
  • Counselling Staff
  • Various Clinic Staff
  • Ward Staff

NET cancers are difficult to diagnose. The onset of symptoms takes an average of three to five years, and even then symptoms are often non-specific and vague. Early diagnosis can potentially mean a better prognosis for patients, but at present however, most NET cancers are diagnosed at a later stage, when they have already spread to other parts of the body. In these cases, they can rarely be cured, although the symptoms can often be managed successfully for a number of years.

The symptoms may be mild or non-specific, and are often similar to those of other, more common conditions.

Many doctors are unfamiliar with NET cancers and are therefore less likely to suspect a NET cancer in their initial investigations. NET cancers are difficult to detect for a number of reasons:

Reasons why NETs are difficult to detect

Size: they are often small; some NET cancers can be less than a centimetre in size
Location: NET cancers can occur almost anywhere in the body
Symptoms: these can vary enormously, and some patients have no symptoms at all
Testing: there are many different types of NET cancer, and a series of specialised tests is necessary for a definitive diagnosis

There are many tests and scans available that will provide your doctor with information about the NET, its spread and rate of growth. Some of these tests work by looking for hormonal changes triggered by the cancerous cells, while others look for the tumour itself:

Biopsy

This involves taking a piece of tissue from the suspect tumour and analysing it in the laboratory by a specialist called a histopathologist.

The specialist may review the biopsy sample and give your tumour a 'proliferative index' i.e. a measure of the number of cells in the tumour that are dividing (proliferating). A proliferation index of less than 2% means that the tumour is very slow growing and in general terms, the higher the proliferation index, the higher the risk of the tumour growing

Being able to look at the tumour under the microscope can be the only way to determine exactly what type of NET cancer it is.

Blood tests

You may be asked to have a fasting gut hormone blood test, and blood will also be collected for a range of other tests. Doctors will be looking for certain NET markers, particularly chromogranin A and B. Other examples of hormones and peptides that the doctors may be looking for are pancreatic polypeptides, insulin and gastrin, as well as checking how well your kidneys and liver are functioning.

If the results of this test suggest the presence of a NET, further imaging tests should be carried out to confirm the diagnosis.

Other biochemical tests

Summary of other biochemical tests

Full blood count
Kidney function tests (urea and electrolytes)
Liver function tests
Thyroid function tests
Pituitary hormone screen e.g. adrenocorticotropic hormone (ACTH), prolactin, growth hormones and cortisol
Serum calcium, parathyroid hormone levels (in all pancreatic NET patients, as a simple screening test for MEN-1 syndrome)
NT proBNP

Urine tests

5-HIAA (hydroxyindoleacetic acid) is a substance that is naturally produced and eliminated by the body. Normally, only small amounts are present in the urine. Elevated levels in a urine sample may indicate a NET, although further tests are required to confirm the diagnosis.

You will be asked to avoid certain foods 24 hours prior to, and for 24 hours during, the test. The foods include chocolate, olives, bananas, pineapple and its juice, all tomato products, plums, aubergine, avocado, kiwi fruit, walnuts, brazil nuts, cashew nuts, tea, coffee and alcohol.

For three to seven days prior to the test you will also be asked to avoid several medicines i.e. certain cough, cold and flu remedies, treatments for high blood pressure, the muscle relaxants e.g. diazepam, monoamine oxidase inhibitors and any natural herbal products purchased in health food shops that produce the same effects as these medicines. This is because all these products naturally contain substances that might artificially raise your 5-HIAA levels and give a false test result.

Endoscopy

In this test, a flexible camera, called an endoscope, is used to examine the digestive tract. The tube can be inserted down the back of the throat (gastroscopy) or via the rectum (colonoscopy). In both cases you will be offered sedation.

If abnormal-looking tissues are found during this procedure, a sample can be collected and examined under a microscope. A tissue biopsy like this can be the only definitive test for a NET cancer.

Endoscopic Ultrasound

In this test, an ultrasound is first used to locate suspect areas, before an endoscopy is performed.

Wireless Capsule Endoscopy

This is a non-invasive procedure in which a small capsule containing a video camera is swallowed by the patient. As it passes through the gastrointestinal tract it records the mucosa of the small bowel.

These images are transmitted by a radiofrequency signal to a data recorder attached to the patient's waist. After approximately eight hours the capsule is excreted and the recorded data is downloaded to a computer where it can be viewed, edited and reported.

Scans

There are a number of scans you may be asked to have. Your doctor will request the specific test that he/she requires and this may change depending on the stage of disease and reason for scanning.

Reasons for different scans

Screening
Detecting the primary tumour in a person suspected of having a NET
Assessing the extent of disease
Assessing the response to treatment
Follow-up after an initial diagnosis

Ultrasound Scan

Ultrasound scans use sound waves to build up a picture of the inside of the body. They are completely painless. These scans are usually done in the hospital X-ray department.

The ultrasound scanner has a microphone which gives off sound waves. The microphone is passed over your body and the sound waves bounce off the organs inside your body, and are picked up again by the microphone. The microphone is linked to a computer which turns the reflected sound waves into a picture.

Computerised Tomography (CT) Scan

A CT scanner is a special type of X-ray machine which uses ionising radiation to provide a three dimensional picture of the inside of the body. It can be used to determine the position and size of tumours, and regular scans are useful to find out more about the rate of tumour growth and how the tumour is responding to treatment.

Before the scan, you may be asked to have an injection or drink a fluid containing a dye that shows up on the scan.

Magnetic Resonance Imaging (MRI)

An MRI scan can be used to locate a tumour. Magnetism is used instead of X-rays to produce soft tissue images that can distinguish between normal and diseased tissue.

If a tumour is identified in this way, further tests may be needed to confirm the type of tumour.

Nuclear Medicine

Nuclear medicine involves the use of small amounts of radioactive materials (or tracers) to help diagnose NETs. It differs from X-ray, ultrasound and any other diagnostic test because it looks at the function of the organ, tissue or bone, and determines the presence of disease based on structural appearance.

OctreoScan®

These scans use a body imaging technique. Cells that receive hormonal messages do so through receptors on the surface of the cells. For reasons that are not fully understood, many NET cells possess especially strong receptors; for example, GEP-NETs often have strong receptors for somatostatin, a very common hormone.

The OctreoScan uses a synthetic form of somatostatin, which is chemically bound to a radioactive substance. This is injected via a vein in the arm and then observed 24 hours later using a radio-sensitive scan. These scans can diagnose and locate around 80-90% of GEP-NETs, although further scans, such as PET scans may still be required.

MIBG Scan

An MIBG Scan is a nuclear medicine scan which involves an injection of a liquid radioactive material called iodine -123-meta-iodobenzylguanidine or MIBG. The radioactive material is injected into a vein on the front of your arm at the level of your elbow, or in the back of your hand.

A special gamma camera (a type of scanner), finds or confirms the presence of tumours. These tumours involve specific types of nerve tissues and most commonly include phaeochromocytomas (a rare cancer of the adrenal gland which is located on the top surface of the kidneys) and neuroblastomas (cancers which affect nerve tissue). Neuroblastoma tumours usually begin in the adrenal gland but may also be found elsewhere in the body.

Positron Emission Tomography (PET) Scan

A PET scan is a nuclear medicine imaging technique which produces a three-dimensional image of functional processes in the body. When FDG-18 is injected into your body it travels to places where glucose is used for energy. It shows up cancers because they use glucose in a different way from normal tissue. It will also show up changes in tissues that use

Another type of PET scan is the GA-DOTA- octreotate PET scan, which has been shown to have a higher sensitivity (can see more) for NETs compared to the octreotide scan. Not only is this a more sensitive scan but it is also much quicker than the octreotide scan and results are available in a shorter time.

A PET scan is usually used in conjunction with a CT scan to build up a picture of the size, location and status of tumours. However, PET scanners are very expensive and very few hospitals have one. This means that a patient may have to travel to another hospital for their scan.

Not everybody who has cancer will need to have a PET scan; other types of tests and scans may be more suitable.

Bone Scan

A bone scan looks for changes or abnormalities in the bones. It is usually done in the medical physics department, nuclear medicine department, or X-ray department of the hospital.

A bone scan can look at a particular joint or bone. In cancer diagnosis, it is more usual to scan the whole body.

Echocardiogram

Some NETs can release chemicals called serotonin and tachykinins into the blood stream which can travel to the heart and affect the cardiac valves. These are most likey to be NETs in the small bowel. If you have been diagnosed with a small bowel NET, your doctor may do a blood test called a NTproBNP. If the result of this test is elevated, then he/she will send you for an echocardiogram to examine your heart. This is an ultrasound of your heart and is usually performed by a cardiac technician.

A small amount of sterile water will be injected into a vein and this is pictured as it passes around the heart. Depending on the results you may need two further investigations:

  • Transesophageal echocardiogram
  • Cardiac catheterisation

Further Testing


Further tests

  • Barium enema
  • Bronchoscopy for lung NETs
  • Gastroscopy

There is no standard group of tests. Each patient may need to undergo a different combination of tests. If you have any questions about these tests, please do ask your medical team.

The treatment of a NET cancer depends on the size and location of the tumour, whether the cancer has spread, and your overall health.

These are a complex group of cancers to manage, and ideally, a MDT will work with you to determine the best treatment plan. The MDT will always have several goals in mind as they formulate your treatment plan.

Not all of the treatments described here are suitable for all people with NETs. Treatment plans should always be tailored to the individual.

Treatment Goals

The main treatment goals are listed below:

Main treatment goals

Remove the tumour by surgery; however, if the tumour has spread, this may not be possible
Alleviate symptoms
Control the tumour growth
Maintain a good quality of life for you

Some of the treatments that are used to reduce or stabilise tumour size and alleviate symptoms are discussed below. The availability of treatments varies around the country. To find out more about which treatments are available to you, please speak to your healthcare team.

There are a number of treatment options available:

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.

If the tumour has spread (metastasised), 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 a GEP NET or other type of NET is blocking an organ, such as the bowel, surgery may be helpful to relieve the blockage (obstruction). If the tumour has spread to the liver, surgery can be used to remove the parts of the liver containing the tumour. Very occasionally, a liver transplant may be considered.

Surgery may be used throughout a patient's treatment plan for many reasons, including in combination with other therapies.

Somatostatin Analogues

Somatostatin is a substance produced naturally in many parts of the body. It can stop the over-production of hormones that cause symptoms such as diarrhoea, flushing and wheezing. It is also known to have an antiproliferative effect in NET which means that it may slow down the rate of growth of the tumour. Lanreotide and octreotide are somatostatin analogues i.e. drugs that copy or mimic the action of somatostatin.

Some NETs produce hormones that can cause other symptoms, for example, patients with a carcinoid tumour may have diarrhoea, flushing, and wheezing. You may have different symptoms depending on the type of tumour that you have. These symptoms can be distressing and often affect your quality of life.

The aim of treatment with these drugs is to slow down the rate of growth of the tumour, and to improve the symptoms of diarrhoea and flushing for patients who are experiencing these hormone related symptoms.

Lanreotide

Lanreotide can be given as an injection every 7-14 days or as a long-acting injection every 28 days. The long-acting injection can be administered by a nurse, either in hospital or by a practice nurse.

For some patients who are stabilised on their treatment with lanreotide, it may be possible for the patient, or a relative or friend, to be taught how to give the injection themselves. The injection is given in the upper, outer quadrant of the buttock or, if you are self-injecting, into the upper, outer thigh.

If you are using lanreotide at home it should be kept in the refrigerator, in its original package, at a temperature between 2°C and 8°C; it should not be frozen. Your doctor can arrange with Ipsen for lanreotide to be delivered direct to your home or GP surgery. A team of nurses can also provide support and guidance on the correct way to inject lanreotide.

Octreotide

Octreotide can be given as a short-acting injection two to three times a day, or as a long-acting injection administered by a healthcare professional every 28 days. The short-acting form is injected into the tissue under the skin, either in the upper arm, thigh or stomach. The long-acting form should be administered by a healthcare professional and is injected in the large muscle in the buttock.

Octreotide should be stored between 2°C and 8°C; it should not be frozen.

Side effects

There may be some side effects, but it is important to remember that the following are only possible side effects and do not affect all patients:

Loss of appetite and problems in the gastrointestinal tract (gut) such as nausea, vomiting, abdominal pain and bloating, wind, and upset bowels
Occasional discomfort at the site of the injection
After a period of time some patients may develop gallstones as a result of the treatment but your centre monitors this when you have your regular scans
Short and longer-acting injections can affect blood sugar levels

People with diabetes should discuss this with their nurse or doctor.

If you experience any problems (or feel you may have developed another side effect) please tell your doctor or nurse.

Further information

There are DVDs available for you and the person giving the injections, and a host of other information so please get in touch with the NET Patient Foundation who will be able to provide the information to you. There are also resources for your GP and practice nurse which provide in-depth information about these injections.

Interferon

Interferon is a naturally occurring substance that is produced by the body's immune system during an illness such as a viral infection e.g. flu. It is sometimes referred to as biological therapy or immunotherapy and is used to treat some patients with NETs. Sometimes interferon is given on its own, but quite often it is given as a combination therapy with somatostatin analogues. It may not be a suitable therapy for all NET patients.

Side effects

There may be some side effects, but it is important to remember that the following are only possible side effects and do not affect everyone:

Nausea (feeling sick)
Flu-like symptoms (aching in the joints and raised temperature)
Lethargy (tiredness)
Myelosuppression (reduction in the amount of new blood cells produced by your bone marrow) and low mood

These symptoms usually improve after the first few injections.

The flu-like symptoms are often eased by taking paracetamol approximately one hour before the injection, and administering the injection at night so that any side effects occur whilst asleep. Nausea can usually be treated very successfully with tablets or other methods. You will have a blood count checked regularly so that the amount of new blood cells being produced in your bone marrow can be monitored. It is important that you tell your nurse or doctor about any side effects that you have, so that you can be offered help to alleviate them.

Chemotherapy

Some people may be given chemotherapy e.g. to treat pancreatic and bronchial NETs, and also for some NET tumours which are growing a little quicker than they might normally do.

The histology of your tumour will help determine whether chemotherapy will be appropriate for you or not. If you have chemotherapy the oncology team, who are specialists in this field, will look after you.

Researchers are constantly looking at various chemotherapy regimens for NET patients and this may be discussed with you at your hospital appointment. There are details of the clinical trials on the website www.netpatientfoundation.com

Chemotherapy drugs are normally administered through a cannula inserted in your arm but there are also tablet forms. You can normally receive your treatment as an outpatient, although if it is your first time, you may be asked to stay in overnight just to check that you tolerate the treatment.

The drugs used, and any possible side effects, will be discussed in-depth prior to commencing any treatments and written information will also be given. You will be given many opportunities to ask questions.

You may be given an information and record book from the chemotherapy team. It might be helpful to note down the specific contact numbers for them.

Embolisation

If the tumour has spread to the liver, you may be offered hepatic artery embolisation (HAE). In this procedure, a catheter is placed in the groin, and then threaded up to the hepatic artery that supplies blood to the tumors in the liver. Tiny particles called embospheres (or microspheres) are injected through the catheter into the artery. These particles swell and block the blood supply to the tumour, which can cause the tumour to shrink or even die.

This treatment can also be combined with systemic treatments for people with liver metastases and metastases outside of the liver. It is a procedure that would be done by a specialist called an interventional radiologist. You will be sedated for the treatment.

Sometimes this embolisation process is combined with chemotherapy and called Hepatic Artery Chemoembolisation (HACE), or Transcatheter Arterial Chemoembolisation (TACE), or radiotherapy (Radioactive Microsphere Therapy [RMT] or Selective Internal Radiation Therapy [SIRT]).

Radionuclide Therapy

Radionuclide therapy is also called peptide receptor radionuclide therapy (PRRT) or hormone-delivered radiotherapy. It is sometimes referred to as the 'magic bullet'. This treatment involves a similar strategy as that applied in an octreotide scan, but the dose of radiation is high enough to prevent further tumour growth or even kill the tumour.

Radioactive substances are chemically combined with hormones that are known to accumulate in a NET. This combination is injected, the hormones enter the tumour and the attached radiation will kill the tumour cells.

There are a number of different radioactive agents available:

Examples of radioactive agents

  • 177-Lutetium
  • 90-Yttrium DOTATOC

You will be admitted to hospital for these treatments, but usually you will only need to stay in overnight. You will remain in a lead-lined room for 12-24 hours afterwards and have a scan the following day so the doctor can see if the active agent has been taken up correctly.

The therapy is commonly delivered intravenously through a cannula in the arm. In certain centres it can also be delivered transarterially if the doctor wants to particularly target tumours in the liver. This involves administering the treatment under sedation via an artery (see embolisation).

MIBG Therapy

MIBG is a radioactive substance that can also be given as an infusion. The tumour has the ability to recognise and absorb MIBG.

Prior to MIBG treatment it will be necessary for you to take some tablets, given to you on your arrival in the ward, called potassium iodate. These are used to protect your thyroid gland from the effects of radiation. You will also have to be admitted for this treatment and remain in a lead-lined room due to the radioactivity. Your stay is likely to be longer than overnight, but this will be discussed with you on an individual basis.

Molecular Targeted Therapies

The molecular targeted therapies everolimus and sunitinib are types of treatment which should only be prescribed by a doctor with experience in cancer treatment. There are a number of serious side effects with these treatments and if you experience any of them you should contact your doctor or nurse as you may require urgent treatment.

Everolimus

Everolimus can be used to treat advanced NETs that originate from the stomach, bowels, lung or pancreas. It stops some of the signals in cells that make them grow; this helps to stop the cancer growing or slows down growth. You can take everolimus once a day as a tablet.

Side effects

Lung or breathing problems (very common): new or worsening symptoms, such as cough, chest pain, sudden onset of shortness of breath or coughing up blood. These may be symptoms of a condition called pulmonary embolism that occurs when one or more arteries in your lungs become blocked
Infections (very common): including chest infections, serious infections that are commonly known as blood poisoning, and infections that are usually only seen in patients with organ transplants such as fungal infections, with symptoms such as fever, chills or other signs of infection
Kidney disorders: altered frequency or absence of urination may be symptoms of kidney failure
Swelling mainly of the face and throat, causing difficulty in breathing (symptoms of an allergic reaction) In addition there are some very common side effects including mouth ulcers, swelling of your arms, hands, feet, ankles or other parts of your body, anaemia, blood clotting problems, nausea, vomiting, diarrhoea, loss of appetite, disturbance of sense of taste, feeling weak or tired, rash, dry skin, itching, discolouration of nails, nose bleeds, high blood sugar, cholesterol and triglycerides, fever, weight loss and headache. It is important to discuss any side effects with your doctor.

 

Sunitinib

Sunitinib is used to treat pancreatic NETs that have progressed or cannot be removed with surgery. Sunitinib prevents the activity of a special group of proteins which are known to be involved in the growth and spread of cancer cells. You can take sunitinib once a day as a capsule.

Side effects

Heart problems: tiredness, shortage of breath, swollen feet and ankles. These may be symptoms of heart problems that may include heart failure and heart muscle problems
Lung or breathing problems: cough, chest pain, sudden onset of shortness of breath or coughing up blood. These may be symptoms of a condition called pulmonary embolism that occurs when blood clots travel to your lungs
Kidney disorders: altered frequency or absence of urination which may be symptoms of kidney failure
Bleeding: serious bleeding problem, painful, swollen stomach, vomiting blood, black, sticky stools, bloody urine, headache or a change in your mental status
Tumour destruction leading to a hole in the intestine; tell your doctor if you have severe abdominal pain, fever, nausea, vomiting, blood in your stool, or changes in bowel habits Other very common side effects may include reduction in the number of platelets, red blood cells and/or white blood cells, high blood pressure, mouth pain/irritation, mouth sores/inflammation/dryness, taste disturbances, burning or painful sensation in the tongue, upset stomach, nausea, vomiting, diarrhoea, inflammation of the digestive tract lining, excessive gas in the stomach or intestine, constipation, abdominal pain, loss/decrease of appetite, loss of strength and weight loss. In addition there may be yellow skin/skin discoloration, hair colour change, hair loss, rash on the palms of the hands and soles of the feet, blisters, rash, dryness of the skin, extreme tiredness, headache, nose bleeds, pain in arms and legs and rapid tissue swelling caused by fluid under the skin. It is important to discuss any side effects with your doctor.

Other Treatments

Radiofrequency Ablation

Radiofrequency Ablation (RFA) is used if there are relatively few secondary tumours. A needle is inserted into the centre of the tumour and a current is applied to generate heat, which kills the tumour cells.

Cryoablation

This is similar to RFA; a substance is inserted into the tumour, which it kills by freezing the cells.

Percutaneous Alcohol Injection

Pure alcohol is injected into cancer cells in the liver. It works by dehydrating the tumour cells.

External Beam Radiotherapy

External radiotherapy is normally given as a series of short, daily treatments in the radiotherapy department, using equipment similar to a large X-ray machine called a linear accelerator (LinAc). Each treatment is called a fraction. Giving the treatment in fractions ensures that less damage is done to normal cells than to cancer cells.

Selective Internal Radiation Microsphere Therapy

Selective Internal Radiation Microsphere Therapy (SIRT) is a new way of using radiotherapy to treat liver cancers that cannot be removed with surgery. SIRT is internal radiotherapy and is also called brachytherapy.

Your doctor inserts a thin tube called a catheter into the hepatic artery. This is the main artery that supplies blood to the liver. They then send tiny beads called microspheres down the catheter and they get stuck in the small blood vessels around the tumour. The microspheres contain a radioactive substance which give a dose of radiation to the tumour. The radiation from the microspheres damages the blood supply to the tumour, so it cannot get the nutrients it needs. This process is sometimes called radioembolisation. The radiation also damages the DNA of the cancer cells, which stops them growing.

Ongoing Research

Research is ongoing into new treatments. Please check the NET Patient Foundation website for the latest information.

Summary

Because of the rarity of these tumours not all doctors and local hospitals will have the full expertise to deal with this type of cancer.

Some specialist NET centres will only have access to some treatments and so you may find that if a treatment has been agreed, you will be referred to a different hospital to get that treatment.

If required, information can be obtained by your local hospital from the centres that regularly treat people with NETs.

There is also an organisation comprising specialist doctors and nurses from the UK and Ireland which has been set up to discuss the best management for NETs. It is called UKINETs. This group has produced guidelines providing information about the best way to look after people diagnosed with a NET.

You should be given information which will help you and your family make rational choices about the various treatments options. You should not only be informed about the treatment options, but you should also be given the opportunity to be involved in decisions about your own care, at all stages of the cancer journey.

Funding Issues

There may be a situation where you have to wait to commence a treatment, or your doctor has to apply for funding for a treatment that he has recommended for you. This is not an unusual situation when you have been diagnosed with a less common cancer.

Although most cancer treatments are available on the NHS, some are not. If your treatment has been recommended but is not routinely available, this does not mean that the NHS will not fund it for you, but they may need to gain further information on your individual case. This can sometimes seem a lengthy process, and can be a worrying time. Do keep in contact with your healthcare team if you are faced with this situation, as they can help you keep track of any developments.

If a treatment has been rejected then there are many tools available to help you and your medical team to appeal this decision, so please do contact the NET Patient Foundation for information on getting the treatment you need.

Psychological Issues

For many patients and their loved ones, the period of uncertainty whilst waiting for a diagnosis can be the most frightening time of all.

Less common cancers can be difficult to diagnose, and many people have to undergo a series of tests and hospital visits over a period of many weeks or months. During this process, doctors may offer conflicting opinions, or simply be unable to answer your questions.

Even when a diagnosis has been reached, it is often difficult to know how the cancer will behave, and whether treatment will be effective. Try not to 'second-guess' your diagnosis: imagining a 'worst-case scenario' is bound to be very distressing.

Don't be afraid to ask questions. Try taking a friend or relative with you to appointments, and bring a list of questions with you. If you feel that your doctor or nurse hasn't explained things well, the NET Patient Foundation has a help line you can call: 0800 434 6476.

Use the internet carefully; it can be a fantastic source of information, but not all of it is reliable. It is worth double-checking anything you find out on the web.

If your feelings of anxiety are overwhelming, it may help to talk to a psychologist or counsellor. All hospitals will have a team that can help you to deal with these issues, and there are various other sources of help.

NET Patient Foundation

The NET Patient Foundation is a registered UK Charity providing help, support and education to patients, families and healthcare professionals affected by or working with NET cancer.

It has five main activities:


1. Providing accurate and up to date information for people living with, or affected by neuroendocrine cancers
2. Providing support for patients and others affected by neuroendocrine cancers
3. Supporting research and diagnosis initiatives around the UK
4. Raising awareness of neuroendocrine cancer throughout the UK and Ireland
5. Producing educational tools to support all sectors of the healthcare community, including GPs, general nurses and specialist/practice nurses

It has three main aims:

1. Reduce misdiagnosis at GP and local hospital level
2. Reduce the timescale to a correct diagnosis
3. Increase patient referrals to a multidisciplinary team It is hoped that the NET Patient Foundation will help to provide a much greater feeling of community, support, understanding and empowerment among the UK's diagnosed population.

 

If you would like to make a donation to the NET Patient Foundation, please contact the charity via the contact details below.

Don't forget to check the range of services available at your own hospital/centre. Some offer an excellent array of in house care such as counselling, pain management and nutritional services and complementary medicine. In addition your GP may also offer support services - it's worth checking out.

NET Patient Foundation
Enton Mill, Enton, Surrey GU8 5AH or
Holly House (2nd Floor)
74 Upper Holly Walk
Leamington Spa
Warwickshire, CV32 4JL
T: 0800 434 6476
www.netpatientfoundation.com

Patient Support

The Rarer Cancers Foundation

Unit 7B, Evelyn Court, Grinstead Road
London SE8 5AD
T: 0800 334 5551
www.rarercancers.org.uk

AMEND

Supporting patients with multiple endocrine neoplasia and associated endocrine growths

AMEND Admin Office
The Warehouse
No 1 Draper Street
Southborough
Tunbridge Wells
Kent TN4 0PG
T: 01892 516076
www.amend.org.uk

Macmillan Cancer Support

89 Albert Embankment
London SE1 7UQ
T: 020 7840 7840
Helpline: 0808 808 0000
www.macmillan.org.uk