This section discusses treatment for the rarest type of pancreatic tumours: neuroendocrine tumours (NETs). For information about treatment for more common types of pancreatic cancer, including adenocarcinomas, see early pancreatic cancer and advanced pancreatic cancer.
Your medical team will discuss the best treatment for you based on the stage of the tumour (the site, size and if it has spread); the grade of the tumour; whether the tumour is functioning or non-functioning; your general health; and your preferences.
Pancreatic NETs are categorised as either non-hormone producing (non-functioning) or hormone producing (functioning). About 90% of pancreatic NETs are non-functioning. This section focuses on the treatment of non-functioning pancreatic NETs.
Functioning tumours are very rare and are usually managed by an endocrinologist (specialist in hormone disorders) and surgeon. Most of these tumours are named after the hormone they overproduce, e.g. insulinomas produce too much insulin. Contact Cancer Council or the Unicorn Foundation (see below) for information about how functioning pancreatic NETs are treated.
Neuroendocrine tumours can also affect other areas of the body, including the lungs, kidneys and bowel. For information about these types of tumours, call Cancer Council 13 11 20 or contact the Unicorn Foundation (1300 287 363 or unicornfoundation.org.au).
Surgery for early pancreatic NETs
Surgical removal (resection) of the tumour is the most common treatment for people with early-stage disease who are in good health. The surgeon will aim to remove all the tumour from the pancreas as well as the surrounding tissue. The type of surgery will depend on the size of the tumour and where it is located.
Surgery for early pancreatic NETs is often given with the aim of cure. However, there are risks and potential complications involved in pancreatic surgery. For small pancreatic NETs, observation to assess the growth of the tumour may be preferable to major surgery. Your surgeon will weigh up the benefits and impacts of surgery, while taking into account your general health and your preferences.
As with more common types of pancreatic cancer, surgeries for early-stage pancreatic NETs include:
- Whipple procedure – treats tumours in the head of the pancreas. Also called pancreaticoduodenectomy.
- Distal pancreatectomy – removes tumours in the tail or body of the pancreas and often also removes the spleen.
- Total pancreatectomy – removes the entire pancreas and spleen. This may be the best option if the cancer is large, or in multiple places in the pancreas.
In some cases, you will be given somatostatin analogues (see below) before surgery.
Options for advanced pancreatic NETs
Many pancreatic NETs have spread at the time of diagnosis. This is considered advanced cancer and the main aims of treatment will be to control the disease and relieve symptoms. People with advanced pancreatic NETs can live a long time with the disease.
Surgery to relieve symptoms
Surgery can be used to treat blockages caused by the tumour and to reduce the size of the tumour.
If the tumour has blocked the common bile duct or duodenum (first part of the small bowel), a small tube called a stent can be inserted.
If the whole tumour can't be removed, the surgeon may try to remove some of it. This surgery, called debulking, is not always possible and will depend on the tumour's position and size.
Somatostatin analogues (SSAs)
The body produces a hormone called somatostatin, which controls how organs such as the pancreas release other hormones. Somatostatin analogues (SSAs) are medicines that are similar to somatostatin. They may be used when a pancreatic NET is making too much of a hormone and causing symptoms such as diarrhoea and dumping syndrome. SSAs can slow down the release of the hormone and may also slow down the tumour's growth. The main SSAs used in Australia are octreotide LAR and lanreotide. These are given as monthly injections.
Chemotherapy is the use of drugs to kill or slow the growth of cancer cells. It is rarely used for lower-grade pancreatic NETs, but may be used for advanced pancreatic NETs that are high-grade. It may be given on its own, with SSAs or before peptide receptor radionuclide therapy (PRRT, see below).
Targeted therapy uses drugs that work in a different way to chemotherapy drugs. While chemotherapy affects all rapidly dividing cells and works by killing cancerous cells (cytotoxic), targeted therapy targets specific molecules within cells and often works by blocking cell growth (cytostatic). People with advanced pancreatic NETs may be offered targeted therapies such as sunitinib and everolimus to slow the growth of the tumour. These are given as capsules that you swallow.
Peptide receptor radionuclide therapy (PRRT)
PRRT may be offered to some people with advanced pancreatic NETs, but it is available only in certain treatment centres. You will usually see a nuclear medicine specialist and a medical oncologist.
You may have a dose of chemotherapy before PRRT. For the PRRT, you will be injected with a cell-targeting protein (peptide) that has been combined with a small amount of radioactive substance (radionuclide). This mixture binds to the NET cells and delivers a high dose of radiation that kills or damages them. PRRT is given as a day treatment, with a session lasting around four hours. Most people have four sessions about 8–12 weeks apart.
Treatment for liver tumours
Advanced pancreatic NETs often spread to the liver. The tumours on the liver are called metastases. Treatments to control them include:
Radiofrequency ablation and microwave ablation
Using an ultrasound or CT scan, a needle is inserted through the abdomen into the liver tumour. The needle sends out radio waves or microwaves that produce heat and destroy the cancer cells.
In this procedure, a catheter is inserted into the hepatic artery, which supplies blood to the liver. A chemotherapy drug is released into the artery, along with tiny particles called microspheres, which block the flow of blood into the tumour. This causes the tumour to shrink. The procedure is performed by an interventional radiologist.
Selective internal radiation therapy (SIRT)
Also known as radioembolisation, this is done by an interventional radiologist. The radiologist inserts a catheter into the liver's main artery and then delivers tiny radioactive beads (SIR-spheres) to the liver through the catheter. The beads give a direct dose of radiation to the tumour while causing little damage to normal liver tissue.
The treatment options described for advanced pancreatic NETs are generally considered palliative treatment because their main aim is to improve quality of life by reducing symptoms. Palliative treatment is one aspect of palliative care.
- Pancreatic NETs can be either non-hormone producing (non-functioning) or hormone producing (functioning). About 90% of pancreatic NETs are non-functioning.
- Functioning pancreatic NETs are named after the hormone they produce and treated by both an endocrinologist and a surgeon.
- Small pancreatic NETs are not always treated immediately, but will be monitored for changes.
- The main treatment for early-stage pancreatic NETs is surgery to remove the tumour. The operation may be a Whipple procedure, distal pancreatectomy or total pancreatectomy.
- Treatments for advanced pancreatic NETs may include surgery, medicines, and a nuclear medicine treatment called PRRT.
- Surgical treatments to relieve symptoms include stenting for blockages and debulking to reduce the size of the tumour.
- Somatostatin analogues (SSAs), chemotherapy and targeted therapy drugs may be given to slow the growth of the tumour.
- Peptide receptor radionuclide therapy (PRRT) is given by injection and delivers a high dose of radiation directly to the pancreatic NET cells.
- There are also treatments to control cancer that has spread to the liver. These include radiofrequency and microwave ablation, chemoembolisation and selective internal radiation therapy (SIRT).
Expert content reviewers:
Dr Lorraine Chantrill, Senior Staff Specialist Medical Oncology, The Kinghorn Cancer Centre, St Vincent's Hospital, and Honorary Research Fellow, Garvan Institute of Medical Research, NSW; Jennifer Arena, Pancreatic and Neuroendocrine Cancer Care Coordinator, Northern Sydney Cancer Centre, Royal North Shore Hospital, and Northern Cancer Institute, NSW; Dr Michael Briffa, Southern Adelaide Palliative Services, Flinders Medical Centre, SA; Rachel Corbett, 13 11 20 Consultant, Cancer Council Victoria; A/Prof Nick O'Rourke, The University of Queensland, Chairman of General Surgery, Head of Hepatobiliary Surgery, Royal Brisbane and Women's Hospital, QLD; Steve Pendry, consumer; Caley Schnaid, Accredited Practising Dietitian, Northern Cancer Institute and Wellac Lifestyle, NSW; Deane Standley, Consumer.