Treatment for VIPoma

VIPoma is a type of neuroendocrine tumour (NET) that usually starts in the pancreas. More rarely, it can also start in the lungs, bowel and liver.

The treatment you have depends on a number of things. This includes where the tumour is, its size and whether it has spread (the stage).

The first treatment you have is to control the diarrhoea and replace the fluids and minerals you may have lost. You then might have surgery to try to get rid of the tumour. But surgery isn’t always possible. Some VIPomas may have already started to spread when you are diagnosed. Or you might not be well enough to have an operation. You continue to have treatment to help your symptoms if surgery isn’t an option.

Deciding which treatment you need

A team of doctors and other professionals discuss the best treatment and care for you. They are called a multidisciplinary team (MDT).

The treatment you have depends on:

where the VIPoma is and its size
whether it has spread
your general health
whether you have a rare inherited syndrome called multiple endocrine neoplasia (MEN1)

Your doctor will discuss your treatment, its benefits and the possible side effects with you.

You are likely to have a clinical nurse specialist (or CNS). They go to the MDT meetings. They can help answer your questions and support you. They are often your main point of contact throughout your treatment.

Surgery

Surgery is the main treatment for VIPomas. There are different types of operations depending on where the tumour is.

Some of these are major operations and there are risks. But if the aim is to try to cure the VIPoma, you might feel it is worth some risks. Talk to your doctor about the risks and benefits of your surgery.

You usually have open surgery. This means your surgeon makes a large cut in your tummy (abdomen) to remove the tumour. During the operation, they might also remove the nearby lymph nodes if they think your tumour is malignant (cancer).

If your VIPoma has spread to the liver, you might be able to have the liver tumour removed at the same time you have the main surgery. Your surgeon may remove just the tumour, or part of the liver too.

For VIPoma that started in the pancreas

You might have surgery to remove:

just the tumour (enucleation)
the narrowest part of the pancreas and the body of the pancreas (distal pancreatectomy)
the whole of the pancreas (total pancreatectomy)
the widest part of the pancreas, the duodenum, gallbladder and part of the bile duct (pylorus preserving pancreaticoduodenectomy or PPPD for short)
the widest part of the pancreas, duodenum, gallbladder, part of the bile duct and part of the stomach (Whipple’s operation)

Remember to click back to return to the NET section. The rest of the information in the pancreatic section will not apply to you.

For VIPoma that started in the bowel

For a VIPoma in your bowel, your surgeon removes the tumour and checks for other tumours. You may need to have part of the bowel removed.

Remember to click back to return to the NET section. The rest of the information in the bowel cancer section will not apply to you.

For VIPoma that started in the lungs

For a VIPoma in your lung, your surgeon removes the tumour and checks for other tumours. You might just have the tumour removed. Or you may need to have part of the lung removed.

Remember to click back to return to the NET section. The rest of the information in the lung cancer section will not apply to you.

Treatments to help with symptoms

There are several treatments to help with symptoms. You might have them before you have surgery, if you can’t have surgery for any reason, or if the cancer comes back after surgery.

The aim of these treatments is to control your symptoms and help you feel better, rather than cure the VIPoma.

Drips to replace fluid and minerals

You usually have a drip into your bloodstream to replace the fluid, potassium and other minerals you might have lost because of the diarrhoea.

Exactly what you need depends on the potassium and mineral levels in your blood, and how dehydrated you are.

Somatostatin analogues

Somatostatin is a protein made naturally in the body. It does several things including slowing down the production of hormones. Somatostatin analogues are man made versions of somatostatin.

You may have somatostatin analogues to try to slow down the tumour and help with symptoms. The most common drugs used are:

octreotide (Sandostatin)
lanreotide (Somatuline)

Octreotide helps to improve the symptoms of diarrhoea in between 8 and 9 out of every 10 people (between 80 and 90%) with VIPoma.

Surgery to remove part of the tumour

Removing part of the tumour can reduce your symptoms. Your doctor will only suggest surgery if they think it’s possible to remove most of the tumour (at least 90%).

Radiofrequency ablation

Radiofrequency ablation (RFA) uses heat made by radio waves to kill tumour cells. You might have this if the NET has spread to the liver.

Trans arterial embolisation (TAE)

You might have this treatment if the NET has spread to the liver.

Trans arterial embolisation (TAE) means having a substance such as a gel or tiny beads to block the blood supply to the liver NET. It is also called hepatic artery embolisation.

You may also have a chemotherapy drug to the liver at the same time. This is called trans arterial chemoembolisation (TACE). But doctors don't know for sure whether adding chemotherapy is better than having embolisation alone for NETs that have spread to the liver.

Embolisation and chemoembolisation work in two ways:

it reduces the blood supply to the tumour and so starves it of oxygen and the nutrients it needs to grow
it gives high doses of chemotherapy to the tumour without affecting the rest of the body

Targeted drugs

Cancer cells have changes in their genes (DNA) that make them different from normal cells. These changes mean that they behave differently. Targeted drugs work by ‘targeting’ the differences that a cancer cell has and destroying them.

You may have 2 types of targeted drugs called everolimus and sunitinib if your VIPoma has started in the pancreas.

Interferon

Interferon is also called interferon alfa. You may have it if the VIPoma has spread to other parts of the body. And other treatments have stopped working.

You may have interferon alone or together with somatostatin analogues.

Radiotherapy

You may have a type of internal radiotherapy called peptide receptor radionuclide therapy (PRRT). Internal radiotherapy means having radiotherapy from inside the body (as a drip into your bloodstream).

PRRT uses a radioactive substance called lutetium-177 or yttrium-90 attached to a somatostatin analogue.

You may have PRRT if:

your NET has spread to other parts of the body
you can’t have surgery
your NET has receptors on the outside of them called somatostatin receptors (you have special scans called octreotide or gallium PET scans to check for this)

Chemotherapy

Chemotherapy uses anti cancer (cytotoxic) drugs to destroy tumour cells. You may have chemotherapy if the NET has spread to the liver or to other parts of your body.

The most common chemotherapy drugs used are:

streptozotocin or temozolomide
fluorouracil or capecitabine
doxorubicin

Liver transplant

You may have a liver transplant if the NET has only spread to the liver and you’re fit and healthy. But a liver transplant might not be possible even if your doctor thinks you can have it. This is because you need a donor liver that is a close match to yours.

A liver transplant is a major operation and it has many risks. It is rarely used as a treatment for NETs.

Clinical trials

Doctors are always trying to improve treatments and reduce the side effects. As part of your treatment, your doctor might ask you to take part in a clinical trial. This might be to test a new treatment or to look at different combinations of existing treatments.