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Neuroendocrine tumours: advances and developments

Professor Martyn Caplin, Consultant Gastroenterologist at The Wellington Hospital, Chairman of the European Neurocrine Tumour Society and former chair of the National Cancer Insitute Neurocrine Tumour Committee, discusses advances and new developments within NETs

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PM: What exactly is a neuroendocrine tumour (NET)?

PC: Neuroendocrine tumours (NETs) originate from cells that have the ability to make hormones and can be found anywhere in the body. A NET develops when these cells grow abnormally with the most common sites including the intestine, pancreas, stomach and lungs.

PM: What are the symptoms?

PC: Many have no symptoms or non-specific symptoms such as abdominal pain. 10 per cent of patients can present with symptoms such as flushing and diarrhoea, which is part of carcinoid syndrome, or recurrent peptic ulcers and diarrhoea as part of Zollinger Ellison syndrome. Some present with sweaty and vague episodes as part of hypoglycaemia and insulinomas symptoms. The problem can be the rather non-specific symptoms, for example flushing in women is often thought just to be due to menopausal type symptoms. Diagnosis is often delayed, as symptoms may sometimes be misdiagnosed, particularly in patients who experience abdominal pain and diarrhoea and are diagnosed with IBS. The average time to diagnosis can be three to seven years from the time of onset of symptoms, meaning that often patients have advanced disease that has spread to other organs such as the liver, bones and lymph nodes at diagnosis of a NET.

PM: How common are NETs?

PC: New cases in the UK are thought to be relatively rare (incidence 6:100,000) but as the tumours are often slow growing, the prevalence in terms of people living with a NET are reported as high as 30:100,000. This means that there are about more than 10,000 people in the UK with NETs in any year. This rate makes them more common then gastric cancer, myeloma and non- Hodgkins Lymphoma. Over 100 patients alone were seen at The Wellington last year for NET related issues.

PM: What tests are available for diagnosis?

PC: In addition to usual blood tests, the most important is plasma chromogranin A and fasting gut hormones. The 24 hour urine 5HIAA is performed for patients who are thought to have carcinoid syndrome. Standard imaging protocols are used including contrast CT and MRI scans and patients often require special nuclear medicine imaging such as the Octreotide scan or (new to The Wellington) the Gallium-68 Octreotate PET Scan, which lights up tumour ‘hot spots’ and is the most sensitive imaging modality.

PM: What is the background to diagnosis and treatment?

PC: There is a large research programme based at The Royal Free Hospital, which is the largest centre in the UK and the first to be awarded a European Centre of Excellence for NETs. The Wellington Hospital is linked to this European Centre of Excellence as a partner hospital, ensuring we have the right standards and that patients are seen by a multi-disciplinary team. All of that expertise is going to be transferred across here and nurses will have a range of specialist training including radiation.

PM: What about the treatments that will be made available?

PC: At The Wellington we will be able to carry out a whole range of treatments. The first line treatment for patients with symptoms or syndrome are the somatostatin analogues, and these intramuscular octreotide LAR or deep subcutaneous injection lanreotide Autogel usually given every 28 days. Somatostatin analogues have more recently been shown to have anti-tumour effects.

We have GI surgeons and hepatobillary/pancreatic surgeons who can resect the primary tumour and also operate on the liver metastasis. We have thoracic surgeons who have specific experience with lung NETs. A percentage of patients with carcinoid syndrome can get carcinoid heart disease, as the hormone levels affect the valves and make them fibrotic. To support this, we are able to perform carcinoid heart valvular surgery here and have cardiologists who specialise in this area.

PM: What are the options for patients who are inoperable?

PC: Besides the anti-hormonal treatment, we occasionally use chemotherapy, mainly for the higher-grade pancreatic, gastric and lung NETs that are advanced and inoperable. With a small minority, we use interferon which stimulates the immune system to fight the tumour cells. There are new, specifically molecular targeted agents (Sunitinib and Everolimus), which block the enzymes that cause the tumours to grow. Then we have radiological procedures such as embolisation (cutting off the blood supply to liver tumours), radiofrequency ablation (burning away tumours in the liver) and we will be able to offer SIRT radioactive beads into the liver.

Additionally, based on the Octreotide scan or Ga-68 Octreotate PET, we can change the imaging isotope for a stronger radioactive beta emitting particle and inject the ‘magic bullet’ targeted therapies, which then bind to the tumour cells and can kill them – using Lutetium-177 or Yttrium-90 Octreotide.

PM: Can you tell us a bit about advances in treatments and the planned new NETs unit?

PC: All patients are discussed in our multidisciplinary meetings as to the best treatment protocol. We have access to the most recent, cutting edge opportunities. For example, recently we published papers on circulating NET cells in the blood stream, which has prognostic implications. We will be able to tell if tumours are growing and isolate tumour cells in the blood stream. We have a whole programme looking at the genomics and isolating DNA from circulating tumour cells. The aim is to have a liquid biopsy where we can repeatedly look for any changes in the genetic profile of the tumour, following different treatments.

The basis of the Unit is that we have this European Centre of Excellence status and we are transferring those world class quality standards and reputation to the private sector. It will be unique with nothing else in the independent sector to match it. It will adhere to the Centre of Excellence guidelines so that we know that we are always treating patients optimally.

As these cases are relatively rare, we will also be supplying information to the primary care and international referrers. We will also have hotlines so people can get hold of the team easily. These are important aspects and undoubtedly a key feature to the success of the new unit.


Professor Martyn Caplin, Gastroenterologist, Tumour, Neuroendocrine, Carcinoid Syndrome, Pancreatic, Gastric, Lung, NETs, European Centre of Excellence

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February 2014
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Professor Martyn Caplin

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