Pancreatic neuroendocrine tumours (pancreatic NETs): symptoms, tests and treatment

Cancer Research UK describes pancreatic neuroendocrine cancers as rare cancers that start in the neuroendocrine cells of the pancreas. Neuroendocrine cells are found in many organs. NHS inform explains that they are similar to nerve cells and make chemical messengers called hormones, which control how different organs work.

There are two key groups of neuroendocrine cancer: neuroendocrine tumours (NETs) and neuroendocrine carcinomas (NECs). Cancer Research UK stresses that they are very different and that it is important to know which one you have. This page is about NETs.

The pancreas is part of the digestive system and lies across the body just behind the stomach. Most non-functioning NETs of the pancreas start in the head of the pancreas, according to Cancer Research UK.

Doctors sometimes group NETs by whether they release abnormal levels of hormones. Cancer Research UK explains that functioning NETs make and release abnormal levels of hormones and non-functioning NETs do not over-produce hormones or cause a syndrome. NHS inform describes the same split.

Cancer Research UK says some people with non-functioning pancreatic NETs have no symptoms and the tumour is found when looking for something else. Where there are symptoms, they are caused by the growth and spread of the tumour.

• Tummy pain.

• Indigestion (dyspepsia).

• Yellowing of the skin and whites of the eyes (jaundice).

• Unexplained weight loss.

• Loss of appetite.

Cancer Research UK says these symptoms are often vague and can also be caused by other more common medical conditions, but that it is important to get them checked out. You usually see your GP first, and they will decide whether you need to see a specialist.

Because the tumours are rare, a doctor may ask you to have tests that check for other conditions first. For functioning tumours the symptoms depend on the hormone; for example, an insulinoma affects blood sugar. NHS inform says that the symptoms of NETs depend on where in the body the tumour is, and that some NETs do not cause symptoms and are found by chance.

Cancer Research UK says tests might include blood tests that check your general health and levels of substances raised by neuroendocrine cancer, a CT scan, an MRI scan, an endoscopic ultrasound scan that can also take biopsies, a PET-CT scan, and radioactive scans such as octreotide, Tektrotyd or gallium PET scans. You may also have a blood test for a rare inherited condition called multiple endocrine neoplasia 1 (MEN1), usually requested by specialist genetic doctors.

NHS inform adds that blood tests may look at chemicals such as chromogranin A and that a biopsy takes a small piece of tissue from the tumour to look at under a microscope.

Grade describes how quickly the cells are dividing. Cancer Research UK says there are 3 grades of pancreatic NET: grade 1 grow slowly, grade 2 at a moderate speed and grade 3 quickly. There are 4 main stages (1 to 4), reflecting size and spread, and treatment depends on grade and stage.

We do not know what causes most neuroendocrine cancers, according to Cancer Research UK. Risk factors for pancreatic NETs include inherited conditions such as MEN1 and Von Hippel-Lindau (VHL), and a family history of cancer.

NHS inform says around 1 in 20 NETs occur in people with a hereditary condition, that NETs may occur at a younger age in these people, and that if you are diagnosed younger or have a family history you may be offered genetic testing. It gives the average age at diagnosis of NETs as around 50 or 60.

A multidisciplinary team (MDT) discusses the best treatment for you, according to Cancer Research UK. It depends on where the NET is and its size, how many tumours there are, whether it has spread, your general health and the symptoms you have. You are likely to have a clinical nurse specialist as your main point of contact.

Watch and wait: because some NETs grow very slowly or not at all for months or years, doctors weigh the risks of surgery against the benefits, and the team may keep an eye on the NET with regular scans. If a NET is smaller than 2 cm and is not causing other problems, Cancer Research UK says surgery might not be recommended.

Surgery: usually the main treatment. Operations named by Cancer Research UK include enucleation (removing just the tumour), distal pancreatectomy, pylorus preserving pancreaticoduodenectomy and the Whipple’s operation. It says some are major operations with risks and that surgery may be suggested even if the cancer cannot be completely removed, to control it for some time and help with symptoms.

Other treatments: if you cannot have surgery, the NET has spread or it has come back, treatments can control symptoms but will not get rid of it. Cancer Research UK lists somatostatin analogues such as octreotide and lanreotide, peptide receptor radionuclide therapy (PRRT), chemotherapy and targeted cancer drugs such as everolimus and sunitinib. For spread to the liver, options include radiofrequency or microwave ablation, surgery, and, rarely, liver transplant.

Cancer Research UK says follow-up appointments check how you are and whether you have problems, and may include blood tests and an MRI or CT scan. How often you are seen depends on the grade of your NET, whether it has spread, the treatment you had and any side effects. It encourages you to contact your doctors if you have worrying symptoms between appointments.

On outlook, it says the best person to talk to about prognosis is your doctor, and that survival depends on many factors including the type of NET and its stage, so no one can say exactly how long you will live.

Pancreatic NETs are managed in the NHS by specialist MDTs, usually in larger centres with experience of neuroendocrine tumours, after GP referral and diagnostic tests.

Some people also seek a private specialist for faster tests or a second opinion. Because grading, staging and the choice between watchful waiting and surgery are nuanced, it helps to ensure that results from any private tests reach the NHS MDT.