Carcinoid Tumours

What is this?

These are found in as few as 1 in every 100,000 people. The most common site is the appendix but they can also occur in the large bowel especially the rectum, the small bowel, stomach, duodenum, pancreas, biliary tract and bronchus (lung). Often those in the appendix are benign but others are more aggressive and can eventually spread to the liver.

Often the tumours are very small (<2cm> and so often cause no obvious bother to the patient. However once they spread to the liver then a specific set of symptoms occur called the ‘carcinoid syndrome’. These symptoms occur because the tumour releases biologically active chemicals (serotonin, bradykinin etc) into the systemic circulation which previously have been filtered out by the liver. These then produce the symptoms that the patient complains about. Occasionally these symptoms can occur without spread to the liver and this is often found with bronchus carcinoid tumours.

What are these symptoms?

• Flushing occurs in about 80% of patients. This is often spontaneous lasting 2-5 minutes but may be triggered by a meal, emotion, exercise, hot drinks or alcohol. Bronchial carcinoid tumours can produce a flush lasting for hours. The flush occurs mainly over the head and neck but also over the skin of the back, abdomen and palms.The flush tends to redden the skin. Some develop watery eyes and their face turns a purplish colour whereas others turn bright red during the flush. The strongest flush reaction can be in the morning when having breakfast.
• Diarrhoea occurs in about 85%. This is usually watery and may occur with the flushing. This can occur sometimes as much as 20 times per day!
• Abdominal pain alone with or without diarrhoea occurs in 40% of patients.
• Asthma-like episodes where the patient wheezes associated with flush attacks occur in about 20%.
• Heart problems occur in up to 70% of patients.This produces shortness of breath on least exertion as well as swelling on the legs. The cause is a thickening of the two right sided heart valves (pulmonary and tricuspid) making them malfunction.
• Hormones released from the carcinoid may cause specific diseases such as multiple stomach and duodenal ulcers, hypoglycaemia or even the rare Cushing.s Syndrome.
• Miscellaneous symptoms can occur. Bleeding from the gut, muscle weakness (myopathy), arthritis, and severe depression.

How is it diagnosed?

• This is usually confirmed by finding excess of the compound 5 HIAA in a 24hr urine collection. However excretion is variable and may have to be repeated twice more before the condition is confirmed. Also certain foods such as bananas, avocado, pineapple, walnuts, coffee and chocolate can give falsely raised levels of 5HIAA and so the specialist may ask the patient not to eat these while the urine is collected.
• Chromogranin A in the blood is elevated in carcinoid and may also be measured.

Both the blood and urine tests are used to follow progress of treatment.

What other tests might I require?

• Ultrasound investigation of the abdomen especially the liver, CT scan of lung and abdomen or MRI scan may all be required.
• Sometimes a special test called ‘angiography’ of the gut blood vessels may be required to follow the blood supply to the tumour and so find where it is situated.
• Barium follow through and barium enemas are often done to search the gut. This is where you swallow a liquid and Xrays are taken as the liquid passes along the small bowel (barium follow through). In barium enema the liquid is passed up the back passage and Xrays taken.
• Gastroscopy of the stomach, sigmoidoscopy of the back passage and colonoscopy of the bowel. All procedures where tubes are passed to visualise the stomach gut and bowel.
• Bronchoscopy may be required for lung carcinoids. Here the specialist looks down the airways in your lung with a special flexible tube.
• Ultrasound of the heart (called Echo) to detect the heart lesions
• Hormone tests may be measured in your blood.
• Octreotide scan. This can often detect small carcinoids especially liver spread.Another scan often used is MIBG.
• Bone scan may be required if the carcinoid has invaded the bones
• Sometimes a laparotomy is required to find the tumour. Here the surgeon physically looks for the tumour inside the abdomen.

This may involve surgery, medicines, chemotherapy and isotopes.

• Surgery. Removal of a bronchial carcinoid often cures that condition. Likewise small appendix tumours are curable by surgery as these are often benign. Tumours in other parts of the body will also be removed but difficulties may arise if they have spread especially if the liver is involved. If all the liver is invaded by tumour then this cannot be surgically removed but if only one part is involved then surgical removal of that area may be possible.
• Liver treatment. If surgical removal of the tumour is not possible then the liver tumours may be treated by the technique of ‘hepatic artery embolisation’. In this technique either gelfoam or a steel coil is injected into the artery feeding the tumour in the liver blocking its blood supply and causing the tumour to die out. Unfortunately some of the tumour may live on and then regrow when new blood vessels reform bypassing the blockade after some 6 to 12 months. The procedure may be repeated.. After the technique the patient can feel very unwell for up to 3 weeks with sickness, vomiting, fever and abdominal pains.
• External radiotherapy is sometimes used for bone pain but is not very successful elsewhere for tumour destruction.
• Octreotide. The symptoms can be considerably relieved by the medicine octreotide. Octreotide stops the release of the chemical and hormones producing the symptoms with improvement in over 70% of patients but regrettably not in all. Octreotide has to be given by injection under the skin 3 times per day. Lately long acting forms have become available. Somatostatin LAR is injected every 2 to 4 weeks while lanreotide is injected every 1 to 2 weeks. Nevertheless the effect of these medicines on tumour growth is not as good as their effect on symptoms. So whereas the patient may have profound relief of symptoms, the tumour may go on growing requiring other therapies.
• Alpha interferon.Another agent which is used in those who do not respond to octreotide or do so poorly is alpha interferon. This is given by injection 3 times per week and is successful in half the patients where it is used but less than 10% show any slowing or shrinkage of the tumour. Alpha interferon has side effects which sometimes are such that they outweigh the benefit. The patients can feel as if they have permanent flu symptoms and chronic fatigue.
• Chemotherapy. To try and reduce tumour growth chemotherapy may be used. Regrettably only 10 to 30% of patients show major shrinkage of the tumours (that is <50%). The drugs streptozocin, 5-fluorouracil and doxorubicin may be used.
• Radio-isotope MIBG therapy. High radioactive MIBG therapy may also be effective. Prior to this therapy a MIBG scan will be done to find out if the tumours take up the MIBG. If they do then it is possible to load the MIBG with high radioactivity and so target the tumours . Regrettably this is not always effective and may need repition. MIBG also enters the thyroid and so try and prevent this the patient is asked to take potassium iodide. Nevertheless the patient should be aware that an underactive thyroid often occurs requiring thyroid hormone repacement medicine for life.
• High radioactive labelled octreotide therapy. If the tumour takes up octreotide on scanning then this new therapy may be useful and is increasingly being used instead of MIBG.
• Other treatments. Ondansetron is another medicine which can be helpful and occasionally the specialist will prescribe other drugs to help specific symptoms.Symptoms of diarrhoea may be helped by loperamide. The hypoglycaemia controlled by diazoxide, Cushings Syndrome by adrenalectomy, acid overproduction in the stomach by medicines such as ranitidine and omeprazole or similar medicines. The specialist will advise.

You will need life long follow up by the hospital specialists. Often you will be seen by quite a few specialists namely the endocrinologist, surgeon, radiotherapist, medical oncologist and radiologist. Your GP will receive from everyone communication detailing your therapy that you require day to day.

Bronchial and appendix carcinoids are usually cured by surgical removal,. The other gut carcinoids with treatment lead to a marked improvement in over 70% of patients but regrettably the therapy is palliative not curative. Nevertheless the prognosis is such that over 40% have a reasonable quality of life for 5 years and some for considerably longer.

For further information you might wish to visit the following web site:
www.carcinoid.org

For information you might wish to contact the new support group for carcinoid patients:
Living with Carcinoid (Tel: 0208 398 3931)

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Ó NHS Tayside; 2006; version 1.0

Disclaimer; no liability whatsoever is accepted for information given and all such information, especially with regard to drug usage (UK version provided), must be checked with a person’s health provider.