What are pancreatic neuroendocrine cancers?
Pancreatic neuroendocrine cancers develop in the 
of the pancreas. Your healthcare team might call them pancreatic neuroendocrine neoplasms (NENs). But neuroendocrine cancer and neuroendocrine neoplasm mean the same thing.
There are different types of cancer that develop in the pancreas. The most common type are pancreatic cancers. These start in cells that produce pancreatic digestive juices.
Pancreatic neuroendocrine cancers are much rarer. They start in the endocrine cells of the pancreas. This is where and other are made and released into the bloodstream. They are also called pancreatic neuroendocrine tumours (PNETS) or islet cell tumours.
There are 2 key groups of neuroendocrine cancer:
- neuroendocrine tumours (NETs)
- neuroendocrine carcinomas (NECs)
NETs and NECs are very different. So it is important to know which one you have. Talk to your doctor or specialist nurse if you are not sure.
There are different types of pancreatic NETs. The 2 main groups are:
- functioning pancreatic NETs
- non functioning pancreatic NETs
Pancreatic neuroendocrine cancers are rare. The treatment and is different to the more common type of pancreatic cancer. So it is important you know what type you have.
The pancreas
The pancreas is part of our .
The pancreas is quite high up in the tummy (abdomen). It lies across your body where the ribs meet at the bottom of the breastbone, just behind your stomach. It is about 6 inches long (15 centimetres) and shaped like a leaf.
The pancreas has 3 parts:
-
the wide part is the head
-
the thin end is the tail
-
the part in the middle is the body
The pancreatic duct is a tube that collects the digestive juices made by the pancreas. It carries them into the first part of the bowel (duodenum).
The pancreas makes digestive juices. It also makes different types of hormones such as insulin. The part of the pancreas which produces hormones, including insulin, is called the endocrine pancreas. The part that makes digestive juices is the exocrine pancreas.
How common are pancreatic neuroendocrine cancers?
Pancreatic neuroendocrine cancers are rare pancreatic cancers. Around 400 people are diagnosed with pancreatic neuroendocrine cancers in England every year.
There are different types of cancer that can develop in the pancreas. Only 1 to 2 out of every 100 pancreatic cancers (1 to 2%) are a pancreatic neuroendocrine cancer.
Neuroendocrine cancers can start in different parts of the body. Around 8 out of every 100 neuroendocrine cancers (around 8%) start in the pancreas.
Risks and causes of pancreatic neuroendocrine cancers
A risk factor is anything that increases your risk of getting a disease. But having one of these risk factors doesn’t mean that you will definitely develop a cancer.
We don’t know what causes most neuroendocrine cancers. But there are some risks factors that can increase your risk of developing it. Having one of these risk factors doesn’t mean that you will definitely develop a cancer.
The risk factors for pancreatic neuroendocrine cancer include:
- inherited conditions such as
multiple endocrine neoplasia 1 (MEN1 ) and Von Hippel-Lindau (VHL ) - having a family history of cancer
Symptoms of pancreatic neuroendocrine cancer
Symptoms are usually caused by:
- the growth of the cancer in your pancreas
- cancer spreading to another part of your body
- hormones made by the cancer
You might hear the terms ‘functioning’ and ‘non functioning’ neuroendocrine tumour (NET). This refers to whether the cancer over produces hormones. These hormones cause symptoms.
- Non functioning NETs don’t over produce hormones or cause a syndrome.
- Functioning NETs over produce certain hormones. This causes a group of symptoms called a syndrome.
We have a page for each type of pancreatic NET. You can read more about the symptoms of each type on the relevant page.
Cancer in your pancreas can cause some general symptoms. These might include:
- tummy pain
- unexplained weight loss
- loss of appetite
- indigestion (dyspepsia)
- yellowing of the skin and whites of the eyes (jaundice)
These symptoms could be due to a pancreatic neuroendocrine cancer. But they can also be caused by other medical conditions. It’s important to get them checked out by your doctor.
Getting diagnosed with a pancreatic neuroendocrine cancer
You have some tests to diagnose a neuroendocrine cancer of the pancreas. This might include blood tests and scans. Your GP can refer you to see a specialist.
If your doctor suspects cancer, you have tests to check the type of cancer you have. Tests also show the size of the cancer and whether it has spread. This helps your doctor plan your treatment.
Tests might include:
- blood tests - these check your general health and check levels of substances raised by neuroendocrine cancer
- a
CT scan - an
MRI scan - an
endoscopic ultrasound scan of your tummy to look at your stomach, pancreas and bile ducts, and to take biopsies - a
PET-CT scan to show the size of the cancer and whether it has spread - radioactive scans – these include
octreotide scans or Tektrotyd scans, and gallium PET scans - urine tests to check levels of substances raised by neuroendocrine cancer
You might have other tests, depending on your symptoms. Your doctor can tell you which tests you need.
Survival (prognosis)
The best person to talk to you about your outlook (prognosis) is your doctor. Not everyone wants to know. People cope differently with cancer and want different information.
Survival depends on many factors. So no one can tell you exactly how long you will live. Your doctor might be able to give you some guide, based on their knowledge and experience.
Your prognosis will depend on the type of pancreatic neuroendocrine cancer you have. And how far it has spread (the stage).
Grades, types and stages of pancreatic neuroendocrine cancer
The tests and scans you have give information about the type, and grade. This helps your doctor decide which treatment you need.
There are 2 main groups of pancreatic neuroendocrine cancer:
- pancreatic neuroendocrine tumours (NETs)
- pancreatic neuroendocrine carcinoma (NECs)
Types of pancreatic NETS
There are many different types of pancreatic neuroendocrine tumours (NETs):
- Glucagonomas come from cells that make glucagon.
- Gastrinomas come from cells that make gastrin.
- Insulinomas come from cells that make insulin.
- Somatostatinomas come from cells that make somatostatin.
- VIPomas come from cells that make vasoactive intestinal peptide (VIP).
Pancreatic neuroendocrine carcinomas (NECs)
NECs are poorly differentiated cancers. This means the cancer cells look very different to normal neuroendocrine cells. They are fast growing and have often spread to other parts of the body at diagnosis.
