Lung cancer is a significant health concern worldwide. Within the diverse landscape of different types and morphologies, lung neuroendocrine tumours (NETs) represent a rare but important subgroup.
Lung cancer is often associated with the more common non-small cell lung cancer (NSCLC) variants including adenocarcinoma, squamous cell carcinoma, and large cell carcinoma. Lung NETs like small cell lung cancer (SCLC) have their unique characteristics and treatment considerations.
Each patient’s experience is unique, and there are medical professionals and support networks available to provide guidance, care, and compassion throughout the process.
On this page, we will explore everything there is to know about lung NETs, providing information and offering support for Australians affected by this condition. If you have specific questions about your condition, it’s always best to talk to your care team.
Lung neuroendocrine tumours are a group of neoplasms that originate from neuroendocrine cells within the lung. These cells play a crucial role in regulating various physiological functions in the body, such as hormone secretion and nerve signalling.
Lung NETs are classified into different subtypes, including typical carcinoid, atypical carcinoid, and small cell carcinoma, with varying characteristics and treatment approaches.
These are slow-growing tumours that account for the majority of lung NET cases. They tend to have a favourable prognosis when diagnosed at an early stage.
The 5-year survival rate for typical carcinoid tumours, when diagnosed at an early stage (localised), is estimated to be around 85% to 95%. However, when diagnosed at a later stage with regional or distant spread, the survival rate may be lower.
Atypical carcinoid tumours are also slow-growing but are considered more aggressive than typical carcinoids. They are associated with a slightly higher risk of spreading to other parts of the body.
The 5-year survival rate for atypical carcinoids can range from 40% to 70%. Like typical carcinoids, the prognosis is better when the tumour is localised at diagnosis.
Small cell carcinoma is the most aggressive type of lung NET. It tends to grow rapidly and is often diagnosed at an advanced stage, making treatment challenging.
The prognosis for small cell carcinoma is generally poorer compared to carcinoid tumours. The 5-year survival rate for small cell carcinoma is often lower, typically ranging from 20% to 30%. This is partly due to the fact that small cell carcinoma is often diagnosed at an advanced stage when treatment options are limited.
It’s important to remember that these are approximate survival rates and can vary based on individual circumstances. Advances in medical treatments, including surgery, chemotherapy, radiation therapy, and targeted therapies, have improved outcomes for some patients with lung NETs. Read more about treatments of lung NETs further down the page.
Large cell neuroendocrine carcinoma (LCNEC) is a rare and aggressive type of lung tumour, with features of both small cell lung cancer (SCLC) and non-small cell lung cancer (NSCLC).
Like other types of NETs and other kinds of lung cancers, the exact cause of lung NETs remains unclear. Studies have shown that several risk factors may increase the likelihood of developing these tumours. Here are some of the most prevalent:
In some cases, a family history of lung NETs or certain genetic syndromes, such as multiple endocrine neoplasia type 1 (MEN1), can elevate the risk.
Lung NETs often affect people in their 40s and 50s, and they are slightly more common in men than women.
Symptoms of lung NETs can vary depending on the subtype and stage of the tumour. Many lung NETS may not have symptoms. Recognising symptoms early can lead to prompt diagnosis and treatment:
The process of diagnosing and staging lung NETs involves several steps to determine the subtype, extent of spread, and the most appropriate treatment approach.
X-rays, CT scans, and MRI scans are invaluable tools for visualising the tumour, and assessing its size, location, and potential spread to nearby lymph nodes or distant organs. These imaging studies provide essential information for diagnosis and staging. Ga68 Dotatate PET and FDG PET which can only be ordered by a Specialist.
A biopsy is a crucial step in confirming the type of lung NET. Tissue samples are obtained through various methods, such as bronchoscopy, CT-guided biopsy, or surgery, and then analysed by pathologists to determine the subtype.
Staging is critical for planning treatment. Lung NETs are staged from I (early stage) to IV (advanced stage), with higher stages indicating a more extensive tumour and potential spread. Staging helps guide treatment decisions and prognosis.
Low grade, typical carcinoid: A limited number of cells are dividing – slow growing. These are also well-differentiated.
Intermediate grade, atypical carcinoid: An intermediate number of cells is dividing. These are also well-differentiated.
High grade, large cell and small cell neuroendocrine carcinoma: A large number of cells are dividing. These are poorly differentiated.
Treatment advancements in the field of oncology are continuous, and it’s essential to consult the most recent medical literature or speak with a healthcare professional for the latest updates on lung NET treatment options. Currently treatment options include:
Surgical removal of the tumour is the primary treatment for early-stage lung NETs, especially for typical and atypical carcinoids. Depending on the tumour’s size and location, different surgical approaches may be used.
If the lung NET is slow growing and is not causing any problems it may be suggested to wait and watch until problems develop. Routine scanning is performed.
Chemotherapy is typically reserved for small cell carcinoma or advanced lung NETs. It involves the use of drugs that circulate throughout the body to target and kill cancer cells. It can help slow down tumour growth and alleviate symptoms.
Some lung NETs exhibit specific molecular changes that can be targeted with precision medicines. These targeted therapies can inhibit the growth of cancer cells while sparing healthy tissues.
For lung NETs that produce hormones (functioning NETs), somatostatin analogues can help control hormone levels, manage symptoms like flushing and diarrhoea, and slow tumour growth.
Special molecules can potentially deliver radiation into neuroendocrine cells which have confirmed somatostatin receptors. This treatment is also known as Peptide Receptor Radionuclide Therapy (PRRT).
Immunotherapy, which enhances the body’s immune response to cancer, is being explored as a potential treatment for lung NETs in clinical trials. It shows promise as a future treatment option.
Radiation therapy may be recommended to shrink tumours before surgery, treat inoperable tumours, or alleviate symptoms in advanced cases. It uses high-energy beams to target and destroy cancer cells.
Treatment for lung NETs is tailored to the individual’s specific case, considering factors like tumour subtype, stage, and overall health. How you respond to one particular treatment compared to another patient may be radically different. With your care team, you can decide on a course of treatment that works for you.
A diagnosis of lung NETs can be overwhelming, but it’s essential to remember that you’re not alone. Support from healthcare professionals, family, and friends is crucial during this time. Here are some tips for living with lung NETs:
Treatment options have broadened over the years to give patients a much longer and greater quality of life. If you’re struggling with your diagnosis, there are plenty of support options available to you and your family.
Here are some key resources and organisations that can provide guidance and compassion to those affected by lung NETs:
Neuroendocrine Cancer Australia is here to assist patients and carers as they manage NETs, including lung NETs. We understand that receiving a NET diagnosis is one of the most life-changing events that can happen to a person.
We offer a free, confidential telephone support and information line with a specialist Telehealth NET nurse to answer any questions you might have. Our NET Nurse Hotline is accessible to all NET patients and their families, and you can attend the services in a variety of ways, including:
Consider joining or seeking out local support groups for NET patients. These groups often provide a safe space for sharing experiences, concerns, and advice with others who understand what you’re going through.
NECA also has a range of patient support groups established all over Australia. Here, patients can access local knowledge and support from peers who have been diagnosed with a NET. Participants are more than happy to answer questions about their experiences with NETs and in the healthcare system.
Australia is at the forefront of medical research, and there may be clinical trials available for lung NETs treatment. Participating in a clinical trial can offer access to innovative therapies and the opportunity to contribute to advancements in cancer care.
Emotional and psychological support with a mental health professional can totally change the way you approach your condition. Psychologists and counsellors can help you and your family cope with the emotional challenges that come with a lung NETs diagnosis.
Remember that you’re not alone in your journey with lung NETs. Reach out to these support resources in Australia to access the information, assistance, and compassionate care you need to navigate the challenges of living with NETs. Your healthcare team can also provide guidance on available resources and support networks in your specific region.
