What are neuroendocrine cancers?

Neuroendocrine cancers are a rare and complex group of cancers that begin in the nerve cells and glands of your neuroendocrine system. The neuroendocrine system makes and releases hormones into your blood stream. These hormones help your body work, like when it digests food.

Neuroendocrine cancers can grow slowly or more quickly. They usually form in the digestive system (gut) and the lungs.

Neuroendocrine cancers are also called neuroendocrine tumours or NETS. They are sometimes known as carcinoids.

What are the symptoms of neuroendocrine cancers?

Many neuroendocrine cancers don't show symptoms early on.

General symptoms include fatigue and loss of appetite.

You may also have different symptoms based on where the cancer is in your body and whether it's releasing hormones into your blood.

Symptoms can include:

• abdominal pain and bloating
• bowel obstruction or bleeding from the anus
• low blood sugar
• nausea or vomiting
• rashes
• coughing up blood
• chest pain

Carcinoid syndrome

Carcinoid syndrome is a group of symptoms that can happen when a neuroendocrine tumour in your lungs or gastrointestinal (GI) tract makes too many hormones.

Carcinoid syndrome symptoms include:

• flushing of the face
• diarrhoea
• wheezing
• shortness of breath

Cancers in these areas don't always cause these symptoms.

People with a neuroendocrine cancer can sometimes have a severe case of carcinoid syndrome caused by stress, general anaesthetic or some treatments. This is called a 'carcinoid crisis'.

What causes neuroendocrine cancer?

Most neuroendocrine cancers don't have a known cause, but there are risk factors that may increase your chance of getting some neuroendocrine cancers. These include:

• some genetic diseases
• conditions such as diabetes or peptic ulcers
• over-exposure to the sun

How are neuroendocrine cancers diagnosed?

Neuroendocrine cancers can be difficult to find. If your doctor is concerned you have a neuroendocrine cancer, they may:

• ask you some questions about your symptoms
• do a physical examination
• request urine tests to check for high levels of hormones, such as serotonin, which are commonly released by neuroendocrine cancers
• request blood tests to check for high levels of the protein chromogranin A, which is released by some neuroendocrine cancers

You might have a test where a camera is used to look in your lungs (bronchoscopy), or bowel (endoscopy). Also imaging scans to look for the tumour and to check whether it has spread. A sample of tissue (a biopsy) may be taken from the suspected tumour to test if it's a neuroendocrine cancer.

How are neuroendocrine cancers treated?

There are several ways to treat neuroendocrine cancers. Your specialist will discuss the best treatment option for you.

You might have one treatment or a mix of treatments, like:

• surgery — where all or part of the cancer is removed
• chemotherapy — which can be used either alone or together with radiation therapy
• radiation therapy (radiotherapy) — which is used to kill or damage the cancer cells
• peptide receptor radionuclide therapy (PRRT) —a type of radiation therapy used to control symptoms and growth of the tumour
• liver-directed therapy — which uses heat or chemotherapy to kill cancer that has spread to the liver

Other treatments include:

• somatostatin analogues —injections given every month to help lessen your symptoms or to control growth of the cancer
• targeted therapy — medicines are used to focus on certain parts of cancer cells and stop the cancer growing
• immunotherapy — this helps your body's immune system to fight the cancer and is currently being studied in neuroendocrine cancers

You may not need treatment right away if you have a slow-growing cancer that isn't causing any symptoms. Instead, your doctor will monitor you. This is also called active surveillance, which means watching and waiting.

Living with neuroendocrine cancer

It may have taken you some time to find out that the symptoms you've noticed are related to neuroendocrine cancer. You may also need some time to get used to this news.

Consider mind-body techniques, such as relaxation, meditation, art therapy or mindfulness to help manage your health.

Alcohol and some foods can trigger diarrhoea and flushing caused by carcinoid syndrome. So it may be helpful to see a dietitian who can help you adjust your diet.

Resources and support

• For general information about neuroendocrine cancers, visit the Cancer Council Victoria website.
• Neuroendocrine Cancer Australia website has factsheets about neuroendocrine cancers.
• Neuroendocrine Cancer Australia has support nurses can be contacted on 1300 287 363.

You can also call the healthdirect helpline on 1800 022 222 (known as NURSE-ON-CALL in Victoria). A registered nurse is available to speak with you 24 hours a day, 7 days a week.

Languages other than English

The Cancer Council has guides about neuroendocrine cancers in languages other than English.

NeuroEndocrine Cancer Australia has videos on neuroendocrine cells and carcinoid syndrome in Mandarin and Arabic.

International Neuroendocrine Cancer Alliance has information in many languages.

Information for Aboriginal and/or Torres Strait Islanders

Cancer Council NSW provides information about cancer for Aboriginal and/or Torres Strait Islander peoples.