Treatment Options

Individuals diagnosed with Neuroendocrine Cancer often undergo surgery to remove the tumour / tumours. The specific objectives of surgery depend on various factors, including the type of neuroendocrine cancer, its location in the body, its size, and whether it has spread from its original site.

Different types of surgeons may be involved in treating Neuroendocrine Cancer, depending on their expertise, such as endocrine, colorectal, hepatobiliary, pancreatic, and cardiothoracic surgeons.

For optimal outcomes, surgery for neuroendocrine cancer should be performed in medical facilities with dedicated neuroendocrine cancer specialist units, where surgeons collaborate with a multidisciplinary team comprising of anaesthetists, oncologists, gastroenterologists, nurses, radiologists, and other doctors experienced in managing neuroendocrine cancer cancers.

There are several types of neuroendocrine cancer surgery available:

Curative Surgery: This approach is employed when the cancer has not metastasised beyond its original organ or area. If the tumour can be removed entirely with a clear margin of healthy tissue surrounding it, the surgery may potentially cure the cancer, making further treatment unnecessary. Nevertheless, a follow-up plan will be established to monitor progress after surgery and for any signs of recurrence.

Palliative Surgery: In cases where the tumour has spread extensively or grown too large to be entirely removed, surgery may be considered to de-bulk the tumour. This helps alleviate symptoms caused by the tumour affecting other organs or producing excessive hormones.

Cardiac and Thoracic Surgery: Patients with pulmonary neuroendocrine cancer may require thoracic surgery, while those with carcinoid heart disease might need cardiac valve replacement surgery.

Perioperative and Anesthetic Management: During the perioperative period or surgery, patients with neuroendocrine cancer may be at risk of a condition called ‘carcinoid crisis.‘ The neuroendocrine cancer specialist is to discuss this potential complication with the surgeon and the anaesthetist prior to the surgery to ensure an appropriate management plan is implemented to reduce potential risks.

Overall, surgery can play a crucial role in the treatment of neuroendocrine cancer neuroendocrine cancer, and decisions regarding the surgical approach are based on the individual patient’s condition, the expertise of the medical team and the neuroendocrine cancer MDT meeting management plan.

Potential side effects of Neuroendocrine Cancer surgery

• Pain and discomfort at the surgical site
• Fatigue
• Infection at the incision site
• Altered bowel movements
• Bleeding or blood clots
• Scarring
• Temporary or permanent damage to nearby organs or tissues, depending on the tumour’s location and extent of surgery

Somatostatin, a hormone produced by the body, plays a crucial role in regulating the release of various other hormones from organs. Somatostatin is a hormone made naturally in the body which controls the release of other hormones (such as serotonin) and chemicals from the stomach, pancreas, and the bowel. Medications known as somatostatin analogues (SSAs) mimic the actions of somatostatin.

SSAs are effective in slowing down tumour growth, as well as reducing the release of hormones from neuroendocrine cancer. They are often used to manage symptoms associated with carcinoid syndromes, such as facial flushing and diarrhoea.

In Australia, the primary SSAs used are octreotide  and lanreotide. Typically administered as monthly injections, these medications may be given more frequently if necessary.

It is essential for patients prescribed SSAs to communicate any side effects to their healthcare providers promptly. Proper management of side effects can help improve quality of life during treatment and ensure better symptom control.

Potential side effects of SSAs for Neuroendocrine Cancer

• Loss of appetite
• Nausea
• Vomiting
• Bloating
• Bowel issues
• Abdominal pain
• Gallstones

Chemotherapy, also known as cytotoxic therapy, works by targeting and destroying rapidly dividing cells, including cancer cells. These drugs circulate throughout the body and can reach cancer cells in various organs. While effective in killing cancer cells, chemotherapy also affects healthy cells that divide rapidly, such as hair follicles and cells lining the digestive tract.

For  neuroendocrine cancer, traditional chemotherapy may be used in cases where the cancer is a higher grade (grade 2 and 3) and has spread extensively (metastasised) and other treatment options are limited. Combination chemotherapy regimens like EC (Carboplatin and Etoposide), FOLFOX (Fluorouracil, Oxaliplatin, Folininic Acid) and CAPTEM (Capecitabine and Temozolomide ) have been used in certain neuroendocrine cancers with some success.

Potential side effects of chemotherapy

• Nausea and vomiting
• Fatigue
• Hair loss (alopecia)
• Decreased appetite
• Diarrhoea or constipation
• Increased susceptibility to infections
• Increase bruising or bleeding
• Anaemia (low red blood cell count)
• Mouth sores
• Changes in taste
• Peripheral neuropathy (tingling or numbness in hands or feet)
• Temporary or permanent damage to organs (e.g., kidneys)

Lutetium-177 Octreotate Therapy (Lutate) is primarily used to treat people with neuroendocrine cancer when other types of treatment, such as surgery or chemotherapy, are not suitable or are ineffective. This may be due to the size, location and number of tumours present. Lutetium-177 Octreotate is a very specific therapy that can only be used when tumours express a large number of somatostatin receptors. Many Neuroendocrine Cancer shows an increase in somatostatin receptors. Other tumours such as head and neck cancers, non-small cell lung cancer, small cell lung cancer and Merkel cell cancer may also express somatostatin receptors. If this therapy is being considered, a diagnostic scan is performed (Gallium 68 PET scan) to distinguish if the tumours are positive for somatostatin receptors.

Peptide Receptor Radionuclide Therapy (PRRT) is a targeted form of internal radiation therapy using Lutetium-177 Octreotate (Lutate), which is primarily used to treat people with neuroendocrine cancer when other types of treatment, such as surgery or chemotherapy, are not suitable or are ineffective. This may be due to the size, location and number of tumours present.

Lutetium-177 Octreotate is a very specific therapy that can only be used when tumours express a large number of somatostatin receptors. Many neuroendocrine cancers show an increase in somatostatin receptors. Other tumours such as head and neck cancers, non-small cell lung cancer, small cell lung cancer and Merkel cell cancer may also express somatostatin receptors. If this therapy is being considered, a diagnostic scan is performed (Gallium 68 PET scan) to distinguish if the tumours are positive for somatostatin receptors.

PRRT has been a game-changer for patients with inoperable or metastatic neuroendocrine tumours. Lutetium-177 dotatate is one of the approved PRRT drugs that have demonstrated significant benefits, improving overall survival and quality of life in patients with advanced neuroendocrine cancer.

Potential side effects of PRRT

• Nausea and vomiting
• Fatigue
• Dry mouth
• Loss of appetite
• Hair loss
• Reduced blood cell counts (anaemia, low white blood cells, or platelets)
• Potential kidney or liver damage, though less common with newer PRRT agents

Liver-directed therapies are designed to target liver metastases specifically, while minimising damage to the rest of the body. These therapies are often used when surgery is not feasible or when the metastases are confined to the liver.

One of the liver-directed therapies for neuroendocrine liver metastases is Transarterial Embolisation (TAE) or Transarterial Chemoembolisation (TACE). TAE involves blocking the blood supply to the tumour, causing its shrinkage, while TACE combines embolisation with the delivery of chemotherapy directly to the tumour site.

Potential side effects of liver-directed therapies

• Fatigue
• Skin rash or changes in skin appearance
• Diarrhoea or other gastrointestinal issues
• Hypertension (high blood pressure)
• Elevated liver enzymes
• Changes in blood counts (e.g., low white blood cells or platelets)
• Risk of blood clots

Selective internal radiation therapy (SIRT)

This is the use of radiotherapy to treat liver metastases that cannot be removed with surgery. It is similar to hepatic artery embolisation. An experienced interventional radiologist will insert a catheter into the hepatic artery supplying blood to the NET tumours and tiny beads containing a radioactive substance will be injected. These interfere with the tumour cell DNA and slow tumour growth.

Radiofrequency ablation (RFA)

This is a treatment for metastatic or primary NET and is done by a radiologist. Using ultrasound or CT guidance, a needle (under local anaesthetic and sedation) is inserted through the abdominal wall and into the liver tumour. Once the needle is localised within the tumour, a generator is used to deliver a rapidly alternating current (radiofrequency energy) producing high temperatures (heat) that destroys the cancer cells (necrosis).

Complementary therapies are supportive treatments, used alongside conventional medical care, to help manage the physical and emotional side effects of cancer and its treatment.

Complementary therapies, also referred to as natural or traditional therapies, can be categorised into three main groups:

‘Natural’ therapies: These encompass herbal and naturopathic compounds, Chinese medicines, homoeopathy, and other similar practices.

Mind-body (mindfulness) techniques: This category includes meditation, relaxation techniques, support groups, counselling, music or art therapy, hypnotherapy, aromatherapy, and more.

Physical therapies: Physical therapies consist of massage, yoga, tai chi, acupuncture, reflexology, Pilates, Alexander technique, and other similar modalities.

It is important to understand that these therapies do not adhere to the same strict regulations as evidence-based medical treatments. Nevertheless, research has shown that over 50% of cancer patients have utilised some form of complementary therapy alongside their prescribed medical treatments. It is recommended you discuss the use of complementary therapies with your specialist.

For numerous neuroendocrine cancer patients, adopting a ‘holistic’ approach to their health proves beneficial. The aim of complementary therapies is to complement conventional treatment and to address dietary, physical, emotional, and spiritual needs, thereby enhancing the patient’s overall quality of life.

In recent years, researchers have been exploring the potential of combination therapies, which involve using multiple treatment modalities simultaneously or sequentially. The rationale behind combination therapies is to enhance treatment efficacy and overcome potential resistance to individual treatments.

For example, combining targeted therapies such as PRRT with chemotherapy has shown promise in treating aggressive neuroendocrine cancer. The combination of targeted drugs and chemotherapy can lead to more effective tumour suppression and prolonged disease control.

In certain instances of neuroendocrine cancers, your healthcare providers might discuss the option of palliative care with you. The primary objective of palliative care is to enhance your overall quality of life by easing the symptoms associated with cancer. It is best to start palliative care early, while you are stable, enabling the team to work with you and your family to design a care plan tailored to your needs.

Palliative treatment is effective in reducing pain and addressing various other symptoms. This form of treatment can involve radiotherapy, chemotherapy, or other targeted drug therapies to effectively manage the condition.