This page is about gastrinoma, a rare type of neuroendocrine tumour that usually develops in the gut (digestive system). There is information about
Gastrinomas are rare tumours that usually start in the gastrin producing cells in the small bowel (duodenum) or pancreas.
Gastrin is a hormone that controls the amount of acid in the stomach. Acid helps to digest food. Gastrinomas produce large amounts of gastrin and this causes the stomach to make more acid. Too much acid leads to ulcers in the stomach and small bowel. The combination of high gastrin levels, too much stomach acid, and severe ulcers is called Zollinger Ellison Syndrome (ZES).
Gastrinomas are very rare. In the UK, only 1 to 3 people in every million develop gastrinomas each year.
Between 6 and 9 out of every 10 gastrinomas (60 to 90%) are cancerous (malignant) and the rest are benign tumours. It may not always be possible to tell if you have a benign or a malignant tumour. The tests you have, such as MRI scans or CT scans, may show if the tumour has spread to the nearby lymph nodes or elsewhere. But it may not be until you have surgery that you know for certain if the tumour is benign or malignant.
Gastrinomas are usually slow growing tumours. Those that develop in the pancreas are most commonly in the head of the pancreas. The head of the pancreas is the end closest to the bowel. Tumours developing in the small bowel (duodenum) are usually small and often less than 1cm. They rarely spread to other organs in the body.
More rarely gastrinomas can develop in the stomach, liver, bile duct or ovary. Even more rarely they can develop in the heart or lung.
Gastrinomas are a type of neuroendocrine tumour. Neuro means nerve and endocrine means hormone producing. Neuroendocrine tumours (NETs) are tumours that develop in cells that are triggered by nerve cells to produce hormones.
Gastrinomas are a particular type of gastroenteropancreatic neuroendocrine tumour (GEP NET). They are also called pancreatic neuroendocrine tumours (pNETs or PETs).
There is a separate page about neuroendocrine tumours.
Symptoms are caused by the increase in the amount of the hormone gastrin that the tumour produces. Some people have more than one tumour. The gastrin causes an increase in the amount of acid in the stomach.
The symptoms include
- Abdominal pain – caused by a stomach or duodenal ulcer
- Indigestion (heartburn)
- Feeling and being sick
- Weight loss
The first test you have measures the amount of the hormone gastrin in your blood. For this test you must not eat anything for about 12 hours beforehand. This test is called a fasting serum gastrin (FSG) test.
You will also have a test called a gastric pH level test to measure the amount of acid in your stomach. This helps to tell whether it is a gastrinoma causing the high gastrin levels or if there is another cause. Your doctor may do this test when you are having an endoscopy. Or you may need to have a tube put into your stomach to get a sample of the acid. The doctor passes a tube called a nasogastric tube up your nostril and down your food pipe (oesophagus) into your stomach. They attach a syringe to the tube and draw some fluid out before removing the tube. They send the fluid to the laboratory for testing.
Your doctor may have prescribed a drug such as omeprazole to reduce your stomach acid production. These drugs are called proton pump inhibitors. If you are taking these drugs, you will have to stop taking them for at least a week before the gastric pH level test.
Other tests you may have include
- CT scan
- MRI scan
- Endoscopic ultrasound – this is the same as an endoscopy but using an ultrasound scan
- PET scan
- Radioactive scan (octreoscan)
An octreoscan is also sometimes called somatostatin receptor scintigraphy (SSRS). You have an injection of a substance called octreotide and then have a scan using a special type of scanner. Octreotide is taken up by some neuroendocrine tumour cells. Doctors can attach a radioactive substance to the octreotide that shows up on the scan.
You may also have a test that combines an X-ray of your blood vessels (angiography) and a test which makes gastrinomas produce gastrin. You have this test in the X-ray department. The X-ray shows up the blood vessels and your doctor injects calcium into each of the main arteries in the gut. This makes any neuroendocrine tumours release gastrin into the blood. Your doctor takes samples 30 seconds after each of the injections of calcium to measure the amount of gastrin in each artery. This can help to find the position of a tumour based on which blood vessel it is close to.
If these tests do not diagnose what is causing your symptoms you may have a biopsy. This is when a doctor takes a small sample of tissue from the area and sends it to the laboratory for examination under a microscope.
You will also have tests to check for MEN-1. This includes blood tests to check your levels of parathyroid hormone, calcium, and the hormone prolactin.
The main treatment for gastrinomas is surgery. But surgery is not always possible. Some cancerous gastrinomas have already spread when they are diagnosed. If you can’t have surgery to try and cure your gastrinoma, you will have treatment to control your symptoms.
The treatment you have depends on
- Whether you have one or more tumours
- Whether the tumour is benign or malignant
- Whether the tumour has spread to other parts of the body
- The position of the tumours in the pancreas or small bowel
- Your general health
- Whether you have a familial cancer syndrome, such as MEN 1
Other treatments you may have include
The type of surgery you have depends on
- Whether you have one or more tumours
- Where the tumour is
- The size of the tumour
If your tumour is in the pancreas you may have surgery to remove
- Just the tumour
- The head of the pancreas – pylorus preserving pancreaticoduodenectomy (PPPD)
- The tail of your pancreas – distal pancreatectomy
If the tumour is in your small bowel, you may be able to have a small bowel resection. This is when the surgeon removes the tumour and part of the small bowel. If there is any uncertainty about whether you have more than one tumour you may have an enterotomy. An enterotomy is when your surgeon opens the small bowel and checks for other tumours. Your surgeon will then decide about the best treatment for you.
Whichever type of surgery you have, if your tumour is cancerous your surgeon will also remove nearby lymph nodes.
These are major operations and there are risks with having this type of surgery. But the aim is to try to cure your gastrinoma so you may feel it is worth some risks. You can read more about surgery for tumours in the pancreas, and possible side effects, on the page about surgery to try to cure pancreatic cancer.
If a malignant gastrinoma spreads to another part of the body, one of the commonest areas it goes to is the liver. You may have surgery to remove tumours in your liver. As this is also a major operation and there are risks, you need to think carefully about the benefits. Will it improve your quality of life enough to make it worth having such a big operation? Your surgeon will talk to you about the risks and benefits and answer any questions you have.
If your cancer has spread to your liver it is sometimes possible to have a liver transplant. Transplants have been carried out in young patients when it was not possible to remove the tumour. A liver transplant is not often possible because you need a donor liver that is a close match to yours. It is also a very major operation with risks.
Treating symptoms of gastrinomas
If surgery cannot remove a gastrinoma or if it comes back after surgery you may have treatment to control the amount of acid the tumour is producing. The aim of this treatment is to control any symptoms you have rather than cure the gastrinoma. You may also have these treatments if surgery is not possible due to other medical conditions.
Treatments may include
If you have a secondary tumour in your liver, your doctor may recommend radiofrequency ablation or trans arterial chemo embolisation (TACE) to reduce your symptoms. Or you may have selective internal radiotherapy (SIRT).
Radiofrequency ablation uses heat made by radio waves to kill the cancer cells in the liver.
TACE is when you have chemotherapy injected directly into the area of the tumour in the liver. You also have an injection of another substance which blocks the blood supply to the tumour. This substance may be a gel or tiny plastic beads and helps to keep the chemotherapy around the tumour.
SIRT is when tiny beads that are coated with a radioactive substance called Yttrium-90 are injected into the blood supply to the liver. This is a type of targeted radiotherapy.
Most of the symptoms caused by a gastrinoma are due to the increase in the amount of acid your gastrin cells produce. If you don’t have any treatment for the overproduction of acid you will develop more serious symptoms. The main treatment is tablets to stop you producing so much acid. These drugs are called proton pump inhibitors (PPIs).
If the PPIs don’t work or stop working you may have drugs called somatostatin analogues, such as octreotide or lanreotide. There is information about somatostatin analogues in the carcinoid section of this website. Carcinoids are another type of neuroendocrine tumour and are also treated with somatostatin analogues.
Research is always more difficult with rare conditions. There are fewer people available to take part in trials, so it takes longer to recruit enough people.
There is research looking into diagnosing and monitoring these tumours. There is also research into treatment with different types of chemotherapy, biological therapies, and targeted radiotherapy. You can read below about
A recent phase 3 trial found that people having everolimus for advanced neuroendocrine tumours (NETs) had a longer time before their cancer started growing again compared to people having a dummy drug (placebo). The average time before the tumour started growing again was 11 months, compared to 4.6 months in people having the placebo.
Another phase 3 trial comparing sunitinib with a placebo for advanced NET found that the average time before the cancer started growing again was 11.4 months, compared to 5.5 months in people having the placebo.
The All Wales Medicines Strategy Group (AWMSG) and the Scottish Medicines Consortium say that sunitinib and everolimus should be available on the NHS. You should be able to have sunitinib or everolimus if other treatments are not working, and you have pancreatic neuroendocrine tumour that cannot be removed with surgery, or has spread to other parts of the body. In England NICE have not looked at these drugs for NET. They may be available through individual funding through your PCT or through the cancer drugs fund if your doctor thinks they may be helpful.
Researchers are also looking into targeted radiotherapy or radio labelled treatment. You may hear this called peptide receptor radio ligand therapy (PRRT). This is a way of getting radiotherapy directly to individual tumour cells.
Cells have receptors that cause changes within the cell when they are triggered. For example, the cell may release more of a particular hormone, or it may divide and grow. PPRT works in a similar way to an octreoscan. By attaching radiotherapy to a substance that attaches to the cancer cell receptors, you can deliver radiotherapy directly to them. It is too early in the research to know how well this treatment will work for gastrinomas.
An international trial called the CLARINET study recently tested whether the drug lanreotide could help to control GEP NETs. It found that the drug could help to control the tumours for some time and help some people to live longer. 204 patients from 48 hospitals in 14 countries took part. They had tumours in the pancreas (44%), midgut (36%), hindgut (7%) and 13% were unknown. The makers of lanreotide have applied in the US and in Europe for the drug to be approved as a treatment for GEP NETs.
More information about research
We need more research to find out which of these new treatments work best and when to use them.
To find out more about neuroendocrine tumour trials go to our clinical trials database and select ‘neuroendocrine tumours’ from the dropdown list.
Coping with a rare condition can be difficult both practically and emotionally. Being well informed about your condition and possible treatments can make it easier to make decisions and cope with what happens.
It can also help to talk to people with the same condition. But it can be hard to find someone with a rare tumour. Check out Cancer Chat – Cancer Research UK’s discussion forum. It is a place to share experiences, stories and information with other people who know what you are going through.
You might also find our general coping with cancer section helpful.
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