Neuroendocrine cancer is a rare and complex disease that affects neuroendocrine cells throughout the body. While they are a less common and complex cancer, neuroendocrine tumours can develop anywhere in the body, and are often found in vital organs, including the lungs, pancreas, gastrointestinal tract, and liver. Neuroendocrine tumours (NETs), often exhibit slow-growing characteristics, making diagnosis challenging in the early stages.
However, treatments are available and have improved through recent advancements in medical research and technology. In this article, we will explore the different treatment approaches for neuroendocrine cancer, with a focus on traditional and emerging therapies.
Getting a positive diagnosis of neuroendocrine cancer can be confronting, overwhelming, and emotional. After getting the results, it’s essential that you take some specific steps to ensure you are well informed and receive the best possible care and support when moving through treatment.
NeuroEndocrine Cancer Australia has a NET Nurse support service and we encourage any patients needing support to contact us.
Here’s what to do to begin on the road to treatment:
The first step is to seek out a specialist experienced in treating neuroendocrine tumours – an oncologist, gastroenterologist, or endocrinologist. For optimal care your case should be discussed at a NET Multidisciplinary Team (MDT) meeting and a management plan developed for you.
Take the time to learn about neuroendocrine tumours, including the specific type you have been diagnosed with.
Don’t hesitate to ask your healthcare team any questions you may have about your diagnosis, prognosis, and treatment options.
Work with your healthcare team to determine the stage of your neuroendocrine tumour. This will help determine what treatment options will work best for you.
Neuroendocrine tumours have various treatment options, depending on the type, location, grade, stage, and other factors. Your healthcare team will discuss potential treatment approaches, which may include surgery, somatostatin analogues, chemotherapy, targeted therapy, or peptide receptor radionuclide therapy (PRRT). Consider the benefits, risks, and potential side effects of each option.
The treatment of neuroendocrine cancer depends on several factors, including:
Some of the primary treatment options for neuroendocrine cancer include surgery, somatostatin analogues, targeted therapy, and chemotherapy.
Individuals diagnosed with NETs often undergo surgery to remove 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 NETs, depending on their specialised expertise, such as endocrine, colorectal, hepatobiliary, pancreatic, and cardiothoracic surgeons.
For optimal outcomes, surgery for NETs should be performed in medical facilities with dedicated NET specialist units, where surgeons collaborate with a multidisciplinary team comprising anaesthetists, oncologists, gastroenterologists, nurses, radiologists, and other doctors experienced in managing NET cancers.
There are several types of NET 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.
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 NETs 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 NETs may be at risk of a condition called ‘carcinoid crisis.’ The NET specialist is to discuss this potential complication with the anaesthetist prior to the surgery to ensure appropriate precautions are taken.
Overall, surgery can play a crucial role in the treatment of NETs, and decisions regarding the surgical approach are based on the individual patient’s condition, the expertise of the medical team and the NET MDT meeting management plan.
Traditional chemotherapy, also known as cytotoxic chemotherapy, 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, traditional 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 tumour is a high grade (grade 3) has spread extensively 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 tumours with some success.
Peptide Receptor Radionuclide Therapy (PRRT) is an innovative and targeted approach that has shown success in treating neuroendocrine tumours, particularly those expressing somatostatin receptors. In PRRT, a radioactive compound is attached to a peptide that binds to somatostatin receptors on the tumour surface. When injected into the bloodstream, the radioactive peptide travels to the tumour and delivers radiation directly to the cancer cells while minimising damage to surrounding healthy tissues.
PRRT has been a game-changer for patients with inoperable or metastatic neuroendocrine tumours. Lutetium-177 dotatate (Lutathera) is one of the FDA-approved PRRT drugs that have demonstrated significant benefits, improving overall survival and quality of life in patients with advanced neuroendocrine tumours.
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.
Lutetium-177 Octreotate Therapy (Lutate) is primarily used to treat people with NETs 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. Most NETs 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
Somatostatin, a hormone produced by the body, plays a crucial role in regulating the release of various other hormones from organs. Medications known as somatostatin analogs (SSAs) mimic the actions of somatostatin.
SSAs are effective in slowing down tumour growth, as well as reducing the release of hormones from neuroendocrine tumours (NETs). 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 LAR 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 the overall treatment experience and ensure better symptom control.
Depending on the metastasis of the NET, there may be some specific organ-related therapies that a patient can access to assist with the spread.
Treatment for neuroendocrine tumour of the pancreas with creon, an enzyme replacement therapy for an affected pancreas.
Complementary therapies, also referred to as natural or traditional therapies, can be categorised into three main groups:
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 NET patients, adopting a ‘holistic’ approach to their health proves beneficial. The aim of complementary therapies is 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 (PRRT) with chemotherapy has shown promise in treating aggressive neuroendocrine tumours. The combination of targeted drugs and chemotherapy can lead to more effective tumour suppression and prolonged disease control.
In certain instances of neuroendocrine tumours, 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.
Aside from its role in slowing the progression of neuroendocrine tumours, 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.
Clinical trials for NETs are medical research studies into new and improved treatments that involve the active participation of patients. Through clinical trials, medical professionals can discern whether a new approach to cancer care surpasses the effectiveness of the status quo. These trials are subject to rigorous regulations to guarantee the significance and dependability of the results obtained.
Inquire with your healthcare team about ongoing clinical trials that may be relevant to your specific type and stage of neuroendocrine tumour. Depending on the stage of your cancer, you may qualify to participate in NET clinical trials in your area. These clinical trials are free-of-charge, and you may be compensated for your time. If you’re interested in entering a clinical trial for neuroendocrine cancer treatment, get in touch with your clinical care team.
Now you’ve received your management plan for the treatment for your neuroendocrine cancer. How do you give yourself the best chances of success in treatment? We’ve compiled some of our best tips to keep in mind as your neuroendocrine cancer treatment advances.
Once you and your healthcare team decide on a treatment plan, you must stick to it. Attend all appointments, follow medication schedules, and inform your healthcare provider of any changes in your condition or side effects experienced.
Rely on friends, family, or support groups to provide emotional and practical support during this challenging time. Connecting with others who have experienced or are currently going through a similar journey can be invaluable.
A cancer diagnosis can be stressful, both physically and emotionally. Take care of your mental wellbeing by engaging in relaxation techniques, meditation, or counselling.
While undergoing treatment, focus on maintaining a healthy lifestyle by eating a balanced diet, getting regular exercise (if possible and approved by your healthcare team), and getting enough rest.
Some treatments for neuroendocrine tumours may cause side effects. Monitor your body’s response to treatment and report any new or worsening symptoms to your healthcare team promptly.
Maintain a record of your medical history, test results, and treatment plan. This information can be helpful in future appointments and consultations.
While coping with a cancer diagnosis is challenging, maintaining a positive outlook can make a significant difference in your journey. Focus on the aspects of life that bring joy and happiness.
Based on the type of care you require, your healthcare team might comprise various medical experts, including:
Every individual’s experience with neuroendocrine tumours is unique, and NET treatment is no different. Your healthcare team will guide you through the treatment process, and you can seek support from various resources to help you navigate this journey. Stay hopeful and take each step with confidence in your ability to manage and overcome the challenges ahead.
Share this course with your healthcare professional so they can enrol in this free online course and learn more about how to diagnose and treat NETs in general practice.