Treatment for neuroendocrine tumours

Surgery is the main treatment for most types of NETs, especially for people with early-stage disease who are in otherwise good health. Surgery usually involves removing the cancer and some healthy tissue around the cancer to ensure the tumour is completely removed. It is important to have your surgery in a specialist centre with surgical and anaesthetic experience with NETs.

The type of operation depends on the size of the tumour and where it is located. For Merkel cell carcinoma the surgery may also involve removing the lymph nodes close to the tumour.

Surgery for early-stage NETs is often given with the aim of cure, although there can also be benefits from removing areas of tumour, even if the cancer has spread (e.g. to reduce the risk of bowel obstruction in small bowel NETs). However, there are risks and potential complications involved in surgery. Your surgeon will discuss the type of operation you may need and the benefits and impacts of surgery.

Surgery for non-functional pancreatic NETs

Early-stage

As with more common types of pancreatic cancer, surgeries include:

Whipple procedure – treats tumours in the head of the pancreas and removes the pancreas, the first part of the small bowel, the gall bladder and bile duct. 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.

Advanced

Surgery can be used to treat blockages caused by the tumour and to reduce the size of the tumour.

Stenting – 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.

Debulking – 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.

More information on these surgeries is available from NeuroEndocrine Cancer Australia.

Download our booklet ‘Understanding Surgery’

The body produces a hormone called somatostatin, which controls how organs release several other hormones. SSAs are medicines that are similar to somatostatin. SSAs can slow down or prevent tumour growth, as well as slow down the release of hormones from NETs. They may be used to help control symptoms associated with carcinoid syndrome such as facial flushing and diarrhoea. The main SSAs used in Australia are octreotide LAR and lanreotide. These are generally given as monthly injections, but may be given more often if required.

PRRT, a form of radiation treatment, may be offered to some people with NETs. You will have PET scans first to assess if you are suitable for this treatment. PET scans will show whether your tumours take up the radiation. This treatment uses a radioactive compound bound together with a small molecule that attaches strongly to NET cells. This allows high doses of radiation to be delivered to specific sites of tumours wherever they have spread throughout the body. The most common form of PRRT is 177 Lu-Dota-octreotate (LuTate) therapy. PRRT is available only in certain specialised treatment centres in each state (generally in metropolitan areas). You will usually see a nuclear medicine specialist and a medical oncologist.

You may have a dose of chemotherapy in tablet form before PRRT as a combined treatment. PRRT is injected into your vein via a cannula, with a session lasting around four hours. Most people have four sessions about 8–12 weeks apart. Re-treatment with PRRT is possible in selected cases.

Chemotherapy (sometimes just called “chemo”) is the use of drugs to kill or slow the growth of cancer cells. You may have one chemotherapy drug, or a combination of drugs. This is because different drugs can destroy or shrink cancer cells in different ways. Your treatment will depend on the grade and type of tumour you have. Chemotherapy is more often given to treat high-grade NETs. Your medical oncologist will discuss your options with you.

Chemotherapy is given through a drip into a vein (intravenously) or as a tablet that is swallowed. Chemotherapy is commonly given in treatment cycles which may be daily, weekly or monthly. For example, one cycle may last three weeks where you have the drug over a few hours, followed by a rest period before starting another cycle. The length of the cycle and number of cycles depends on the chemotherapy drugs being given.

Download our booklet ‘Understanding Chemotherapy’

Targeted therapy uses drugs that work in a different way to chemotherapy drugs. While chemotherapy drugs affect all rapidly dividing cells and kill cancer cells, targeted therapy drugs attack specific molecules within cells and work by blocking cell growth. People with advanced pancreatic NETs may be offered targeted therapies such as sunitinib (Sutent) and everolimus (Afinitor) to slow the growth of the tumour. Everolimus may also be used to treat advanced gastro-intestinal, pancreatic and lung NETs. These drugs are in capsules that you swallow.

Download our fact sheet ‘Understanding Targeted Therapy’

NETs, particularly gastro-intestinal and pancreatic, often spread to the liver. The tumours in the liver are called metastases. Treatments to control these liver lesions may include:

Radiofrequency ablation (RFA) 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.

Transarterial chemoembolisation (TACE) – In this procedure, a catheter is inserted into the hepatic artery, which supplies blood to the liver.
A chemotherapy drug together with tiny particles (called microspheres) is released into the artery, which blocks the flow of blood into the tumour.
This may cause the tumour to shrink. The procedure is performed by an interventional radiologist. Sometimes the injection may involve using microspheres alone without the chemothrapy component, called transarterial embolization (TAE).

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.

PRRT can also be used to treat liver lesions in selected cases.

Radiation therapy (also known as radiotherapy) uses high energy rays to destroy cancer cells, where the radiation comes from a machine outside the body. NETs may be treated with external beam radiation therapy in selected cases, depending on the location of tumours and your symptoms. It may be used:

• if the cancer can’t be removed with surgery
• if the cancer has spread to other parts of the body
• after surgery, to destroy any remaining cancer cells and stop the cancer coming back (such as for Merkel cell carcinoma).

Radiation therapy can shrink the cancer down to a smaller size. This may help to relieve symptoms such as pain. Sometimes chemotherapy is given as well as radiation therapy, for example to treat lung NETs.

Radiation therapy does not hurt and is usually given in small doses over a period of time to minimise side effects.

Download our booklet ‘Understanding Radiation Therapy’

Your doctor or nurse may suggest you take part in a clinical trial. Doctors run clinical trials to test new or modified treatments and ways of diagnosing disease to see if they are better than current methods. Over the years, trials have improved treatments and led to better outcomes for people diagnosed with cancer.

You may find it helpful to talk to your specialist, GP, clinical trials nurse or get a second opinion. If you do take part in a trial, you can withdraw at any time.

For more information visit:

• Australian Cancer Trials
• NeuroEndocrine Cancer Australia
• Australasian Gastro-Intestinal Trials Group (AGITG)
• Neuroblastoma Australia
• Melanoma and Skin Cancer (MASC) Trials: masc.org.au and melanoma.org.au

Download our booklet ‘Understanding Clinical Trials and Research’

Complementary therapies are designed to be used alongside conventional medical treatments (such as surgery, chemotherapy, targeted therapy and radiation therapy) and can increase your sense of control, decrease stress and anxiety, and improve your mood.

Some Australian cancer centres have developed “integrative oncology” services where evidence-based complementary therapies are combined with conventional treatments to create patient-centred cancer care that aims to improve both wellbeing and clinical outcomes.

Some complementary therapies and their clinically proven benefits are listed below:

acupuncture – reduces chemotherapy-induced nausea and vomiting; improves quality of life

aromatherapy – improves sleep and quality of life

art therapy, music therapy – reduce anxiety and stress; manage fatigue; aid expression of feelings

counselling, support groups – help reduce distress, anxiety and depression; improve quality of life

hypnotherapy – reduces pain, anxiety, nausea and vomiting

massage – improves quality of life; reduces anxiety, depression, pain and nausea

meditation, relaxation, mindfulness – reduce stress and anxiety; improve coping and quality of life

qi gong – reduces anxiety and fatigue; improves quality of life

spiritual practices – help reduce stress; instil peace; improve ability to manage challenges

tai chi – reduces anxiety and stress; improves strength, flexibility and quality of life

yoga – reduces anxiety and stress; improves general wellbeing and quality of life.

Let your doctor know about any therapies you are using or thinking about trying, as some may not be safe or evidence-based.

Download our booklet ‘Understanding Complementary Therapies’

Alternative therapies are therapies used instead of conventional medical treatments. These are unlikely to be scientifically tested and may  prevent successful treatment of the cancer. Cancer Council does not recommend the use of alternative therapies as a cancer treatment.

If you have been diagnosed with a NET, both the cancer and treatment will place extra demands on your body. Research suggests that eating well and exercising can benefit people during and after cancer treatment. Eating well and being active can help you cope with some of the common side effects of cancer treatment, speed up recovery and improve quality of life by giving you more energy, keeping your muscles strong, helping you maintain a healthy weight and boosting your mood.

Some people with NETs, especially pancreatic NETs or those who have had bowel surgery, may need specific dietary advice. The symptoms of carcinoid syndrome (facial flushing, diarrhoea) may be triggered by certain foods and drinks, and some vitamin deficiency syndromes may be more common. You can discuss individual nutrition and exercise plans with health professionals such as dietitians, exercise physiologists and physiotherapists.

More information on the nutritional needs of people with NETs is available in the Nutrition and Neuroendocrine Tumours booklet available from NeuroEndocrine Cancer Australia.

Download our booklet ‘Nutrition for People Living with Cancer’

Download our booklet ‘Exercise for People Living with Cancer’

All treatments can have side effects. The type of side effects that you may have will depend on the type of treatment and where in your body the cancer is. Some people have very few side effects and others have more. Your specialist team will discuss all possible side effects, both short and long-term (including those that have a late effect and may not start immediately), with you before your treatment begins.

One issue that is important to discuss before you undergo treatment is fertility, particularly if you want to have children in the future.

Download our booklet ‘Fertility and Cancer’

Common side effects may include:

Surgery – Bleeding, damage to nearby tissue and organs (including nerves), drug reactions, pain, infection after surgery, blood clots, weak muscles (atrophy), lymphoedema.

SSAs – Loss of appetite, nausea, vomiting, bloating, bowel issues such as constipation or diarrhoea, abdominal pain, gallstones, fatigue.

PRRT – Nausea, vomiting, fatigue, short-term hair loss, kidney damage and blood disorders, loss of fertility.

Chemotherapy – Fatigue, loss of appetite, nausea, bowel issues such as constipation or diarrhoea, hair loss, mouth sores, skin and nail problems, increased chance of infections, loss of fertility.

Radiation therapy – Fatigue, loss of appetite, nausea, bowel issues such as diarrhoea, abdominal cramps and excess wind, bladder issues, hair loss, dry mouth, skin problems, lymphoedema, loss of fertility.