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Pancreatic Neuroendocrine Tumours

What is the pancreas?

The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine. The pancreas is a dual functioning gland of the digestive system made up of two types of cells: one produces digestive juices and the other releases hormones that help regulate blood sugar levels

What is a pancreatic neuroendocrine tumour (pNET)?

pNETs are tumours which arise from the cells of the pancreas that release hormones. The tumours can be functional (releasing hormones) or non-functioning (not releasing hormones). pNETs are much less common than pancreatic cancer (which originate from the cells that make digestive enzymes) and the prognosis is much better.

Are pNETs cancerous?

Some pets are cancerous (spread to other sites) and some are not cancerous. It is not known whether non-cancerous pNETs can become cancerous as they are rare tumours (1-2 per100,00 people)

Do pNETS spread?

Yes, roughly half of pNETS spread to the liver.

What are the signs of pNETs?

  • Non-functioning pNETS have few symptoms and may only present once they have spread to the liver.
  • Functioning pNETS produce hormones and these hormones produce symptoms. The symptom depends on the hormone produced, these include;
    • Insulin (Insullinoma) – Insulin stores sugar causing low blood sugar, fast heartbeat, irritability and fatigue
    • Glucagon (Glucagonoma) – Glucagon release sugar causing high blood sugar, rash, diarrhoea and weight loss
    • Gastrin (Gastrinoma)- Gastrin produces stomach acid which causes severe stomach ulcers and diarrhoea

These symptoms can also be caused by conditions that are not pancreatic neuroendocrine tumours. But if you have these symptoms, tell your doctor or nurse about them.

What tests are done for pNETs?

  • Blood tests including a CGA level which may be raised in pNETs.
  • Hormone tests – to check levels of hormones produced
  • Ct scan or MRI scan – to look for tumours in the pancreas or liver
  • Octreotide scan or PET scan – use markers which attach to the tumour to locate small tumours and assess their function.
  • Endoscopic ultrasound biopsy – to sample the tumour for assessment under the microscope.

What is the treatment of pNETs?

Treatment depends on the type of pNET, where it is in the pancreas and whether it has spread. The grade of the tumour also plays an important role (determined on biopsy). Treatment options are discussed at an MDT meeting before recommendations are discussed with patients.

The treatment of pNETS has three main categories

Surgery - Pancreatic surgery can be performed to remove a pNET from the pancreas. A small number of people may also be able to undergo a liver surgery to remove pNETs that have spread to the liver. The type of surgery depends on where the tumour is located (see pancreatic surgery)

Chemotherapy - chemotherapy drugs may be used to try and kill cancer cells or to stop them growing.

Hormone therapy - Hormone therapy (tablets or injections) may be used to block the effects of hormones produced and in some cases to stop tumours growing.

Where can I find more information about pNETS?

More information is available on the Australian rare tumours website

https://www.rarecancers.org.au/directory/102/pancreatic-neuroendocrine-tumors-islet-cell-tumors-

What is the prognosis of pNET?

The prognosis of PNET is better than other types of pancreas cancer. As they are rare cancers and each behave differently it is impossible to give general advice about prognosis. This discussion is best had with your surgeon or oncologist and may depend on the response to treatment.