Carcinoid and neuroendocrine tumours

What are carcinoid and neuroendocrine tumours?

These tumours arise from cells of the stomach, intestine, lung, thyroid or pancreas and usually spread to the liver, which is why the patients are often referred to liver units.

The characteristics of these tumours are that they often produce hormones (chemicals in the blood), which have effects on the whole body, not just the area of the tumour.

The doctors and nurses involved in these tumours have in the past belonged to only one speciality, but it is now recognised that staff from many different backgrounds should be involved in the care of these patients.

At Hampshire Hospitals NHS Foundation Trust’s Basingstoke and North Hampshire Hospital we now have a service where these patients are seen and assessed by several doctors and nurses. This provides a combined opinion from staff of different backgrounds and training.

Who is involved in Basingstoke?

The persons that you may see in the clinic are: Dr John Ramage, Consultant Physician in gastroenterology and hepatology; Mr Myrddin Rees, Consultant Hepatobiliary Surgeon; Miss Welsh, Consultant Hepatobiliary Surgeon; Mr Tim John, Consultant Hepatobiliary Surgeon; and Mrs Barbara King, Neuroendocrine tumour nurse specialist.

Referrals are made through GPs or hospital specialists. Referrals can be through Dr Ramage’s secretary or through Barbara King. Fax/Phone: 01256 456778/01256 313637

Research interests of the clinic include: measurement of quality of life in patients with carcinoid and neuroendocrine tumours; embolisation (blocking of one of the liver arteries) using new forms of chemotherapy; and genetics of carcinoid and neuroendocrine tumours.

Further support

Barbara King can be contacted via email barbara.king@hhft.nhs.uk. She can assist by answering questions/queries for both clinicians and patients. She can also help you to find out further information on neuroendocrine tumours. She works three days a week and will endeavour to answer your questions/queries as soon as she can.

The Wessex Carcinoid Group is a group of clinicians from Basingstoke, Winchester, Southampton, Isle of Wight, Portsmouth, Bournemouth, Poole, Salisbury and the Royal Marsden who meet up every 4-5 months to discuss their own patients who have neuroendocrine tumours.

Each clinician presents their individual case to the group which consists of surgeons, gastroenterologists, endocrinologists, oncologists, radiologists, pathologists, nuclear medicine physicians, specialist registrars and specialist nurses. Scans and reports are discussed and reviewed by all the clinicians and recommendations, comments and advice are given once the case has been discussed and analysed. The outcome varies from a referral from one clinician to another for a scan, surgery or specific treatment such as MIBG, embolisation or Yttrium treatment or it may just be a “wait and see” policy, but the clinicians between them try to ascertain the best possible overall treatment plan.

If you are interested in joining the group then please send an email to gastrohep@btinternet.com with your name, specialty, address and email address and you will be added to the carcinoid group distribution list and notified of any forthcoming meeting.

Information for patients with carcinoid tumours:

Carcinoid tumours, now known as neuroendocrine tumours or apudomas, are unusual tumours which develop in patients for no apparent reason. Currently we have no idea what causes them – they do not seem to develop as a result of toxic agents, infections or drugs – and there is no identifiable association with any particular lifestyle or age.

The primary tumour often develops in the small bowel (small intestine) or appendix but it can occur in many other places such as lung, pancreas, large bowel, ovary or testis. Sometimes the primary tumour is never found, and finding and removing the original primary tumour may not necessarily help once secondaries have occurred.

Sometimes the primary tumour is diagnosed early and is removed without any spread, but frequently it is only diagnosed when spread has occurred to the liver. This is why patients are often seen in liver units, where the disease is diagnosed as secondary liver tumours (or metastases). The tumours in the liver are usually multiple and cause the liver to enlarge slowly. Sometimes, the tumours produce hormones, which are chemical substances that cause rather odd effects on the body.

One of the most common syndromes that this produces is what is termed ‘carcinoid syndrome’, where patients develop severe flushing and sometimes wheezing and diarrhoea also. All these effects are due to a hormone secreted by the tumour within the liver. There are various other substances which can be produced, which can cause such effects as diabetes, duodenal ulcers or hypoglycaemic (low blood sugar) episodes. Any unusual or unexplained symptoms should be reported to one of the doctors or nurses looking after you.

Diagnosis

The tumours in the liver are often recognised by ultrasound scanning and this may be followed by CT scans or MRI scans. Sometimes it is necessary to take a biopsy (specimen) of the tumour. This is usually taken by putting a needle through the skin into the tumour and the sample obtained is sent off to a laboratory for processing. Twenty four hour collections of urine and some special blood samples will often detect whether any hormones are being produced by the tumour. Other methods for finding the primary site may be CT scans of the chest, barium studies of the small bowel and sometimes colonoscopy (examination of the bowel using a camera via the rectum) or upper GI endoscopy (examination of the stomach via a camera through the mouth).

The course of the disease

It is important to emphasize that these tumours, although technically malignant and do spread to other parts of the body, are usually slow growing tumours and some patients may not have any change in the size of tumour even over many years. The tumours are often treated by a variety of different people, which include gastroenterologists, hepatologists, oncologists, endocrinologists and radiologists. Like most diseases that are rare, they are probably best treated by doctors who see the condition frequently.

Treatment

There are a large number of options for treatment; these include doing nothing (known as ‘watching and waiting’ – it is important to know that some patients will have many years symptom-free without any treatment).

• Octreotide injections – these are normally given two or three times a day by the patients themselves. This is usually a short term course until a monthly preparation can be started.
• There are newer monthly preparations that can be given once every 4 weeks (Sandostatin LAR or Lanreotide autogel), which are more convenient and give a stable level of medication in the body.
• Interferon injections – these are usually given three times a week and may cause some side effects such as depression, lethargy and effects on the blood.
• Hepatic artery embolisation. This is a procedure done in xray where a catheter is passed into an artery in the leg and threaded up into the liver and a substance is injected in order to block the main artery to the liver. This is very effective but can produce pain and fevers for some days afterwards and occasionally there can be more serious side effects.

Other more invasive treatments include surgery on the liver (removing one or more secondary tumours from the liver), or removing the primary site by surgery. Obviously these would be discussed with the patient in some detail before they would be considered. Chemotherapy is a further option and can be effective in certain types of tumour.

Other treatments, including forms of targeted radiotherapy (delivering high doses of radiation to the tumours by the “magic bullet” principle), are currently used. These may be referred to as MIBG therapy, Indium or Yttrium-octreotide therapy.

Radiofrequency ablation is another new therapy that consists of putting needles or “probes” through the skin directly into the tumour and passing some radiation through the tumour (a bit like putting a transmitter aerial into the tumour). This i2s often done under general anaesthetic. The results are not clear yet since good trials have not been done, but the treatment may be of some help. Alcohol has also been injected into these tumours with a needle through the skin and this can reduce the size of the tumours in some cases.

For further information about neuroendocrine tumours (carcinoid)

visit the NetPatient Foundation website (formally the Living with Carcinoid site). This site is extremely informative as it is directed at patients and their relatives living in the UK.

Other useful websites are:

• Macmillan
• Carcinoid Link (provided by Novartis Pharmaceuticals)
• Ipsen Ltd
• The Carcinoid Cancer Foundation (a US site – remember medicine in the US is different to that in the UK, not necessarily better).

Charitable fund

There is a charitable fund for carcinoid tumours called the Carcinoid Tumour Fund.

Details are available from Barbara King – Tel: 01256 313637.

12 important facts regarding Neuroendocrine tumours (NETs)

1. NETs affect around 2,000 new patients every year.
2. NETS originate from neuroendocrine cells that are a specialized group of nerve cells (the neuro part) that produce hormones (the endocrine part).
3. NETs affect children and teenagers too. The NET Patient Foundation aims to provide information and support all patients regardless of age.
4. There are at least eight different types of NETs that are named depending on the hormone they produce. Those that don’t produce any hormones are described as non-functioning.
5. Although similar in cell type, investigation and treatment options, each tumour type has a different biology and prognosis, which adds to the complexity of managing the disease.
6. Carcinoid tumours represent at least 55% of all NETs – although on reflection many carcinoid patients often recall vague bowel problems or discomfort, which are signs and symptoms of the tumours.
7. There is a great difference in the sensitivity to treatment and survival between pancreatic NETs and pancreatic adenocarcinoma so the proper diagnosis by a specialist is essential.
8. Insulinomas are a type of NET that originate in the pancreas and secrete large amounts of insulin. Although rare, the symptoms they produce include confusion, sweating, dizziness, weakness and relief with eating.
9. Research has found that 1% of the population develop carcinoid tumours without necessarily knowing about it, and only a sub set becomes malignant. At present no-one knows why certain people develop malignancy and others don’t.
10. Many patients have flushing, bowel problems, abdominal pain and heart conditions as a result of their tumours. These symptoms can be controlled by monthly injections. Carcinoid heart disease is due to the excessive secretion of hormones by the tumour, which causes fibrosis of the heart valves. There is a higher incidence of other cancers in patients with NETs.
11. Only 10 years ago the common thinking was not to do anything with these tumours but with a better and growing understanding of NETs, management is now much more active. Funding is urgently needed for research into new treatments that offer the possibility of prolonging life for this patient group.
12. NETs are known as the quiet cancers because initially they may only cause vague symptoms. As a result these tumours are often not detected until they grow large or spread to other organs and cause other problems. Motto: Be body aware.