Supporting the

Neuroendocrine Cancer Community

Neuroendocrine Cancer of the Lungs

Here, you’ll find a wealth of resources tailored to those affected by lung neuroendocrine cancer. We offer comprehensive information on causes, symptoms, diagnosis, and treatment options. Dive into inspiring patient stories, stay updated on research developments, and access expert insights. Our community is here to support you, providing both emotional guidance and practical advice.

Explore treatment approaches, connect with others who understand your journey, and discover ways to lead a fulfilling life during and after treatment. You’re not alone in this – together, we’re here to educate and support.

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The Facts

Neuroendocrine Neoplasm (NENs) serves as an umbrella term encompassing a cluster of cancers originating in neuroendocrine cells. These cancers might also be denoted as NETs, NECs, or Carcinoids. NENs manifest when neuroendocrine cells deviate from their normal function, undergoing abnormal growth or behaviour.

Two principal types of NEN exist:

1. NETs (neuroendocrine tumours): Characterised as ‘well-differentiated‘, these tumours usually exhibit a gradual to moderate growth pattern.

2. NECs (neuroendocrine carcinomas): Identified as ‘poorly differentiated‘, these tumours tend to grow rapidly.

To simplify matters, we employ the term NENs throughout this page.

Lung NENs

The lungs, a pair of expansive and spongy organs, occupy the chest cavity on either side of the heart.

Each lung is divided into distinct lobes: The right lung comprises three lobes – superior, middle, and inferior – while the comparatively smaller left lung contains two lobes – superior and inferior.

Within the respiratory tract, neuroendocrine cells are naturally present in the lungs. Lung Neuroendocrine Neoplasms (NENs) constitute approximately 20% of all lung cancers.

Presently, five types of Lung NENs have been categorised:

1. Typical Carcinoid (TC): Characterized as low-grade neuroendocrine tumours (NETs).
2. Atypical Carcinoid (AC): Identified as mid-grade neuroendocrine tumours (NETs).
3. Small Cell Neuroendocrine Carcinoma (NEC): A distinct type of high-grade carcinoma.
4. Large Cell Neuroendocrine Carcinoma (NEC): Another variant of high-grade carcinoma.
5. MiNEN or Mixed Cell Carcinoma (MiNEN): This subtype includes neuroendocrine cells within a tumour of a different cancer form.

Spotlight on Lung Neuroendocrine Tumours

Click “Play” to view Professor Was Mansoor’s insightful presentation from the 2020 NCUK Virtual Summit: “Spotlight on Lung Neuroendocrine Tumours – 2020.”


Signs and symptoms may or may not manifest. When they do appear, they can either resemble common lung conditions like asthma or exhibit similarities to more prevalent lung cancers. These symptoms encompass:

  • Persistent Cough: A cough persisting for 2 or 3 weeks, potentially accompanied by worsened phlegm that could contain traces of blood.
  • Recurrent Chest Infections: Frequent occurrences of chest infections.
  • Wheezing and Asthma-like Symptoms: Experiencing wheezing or symptoms reminiscent of asthma.
  • Breathlessness: New, persistent, or worsening breathlessness.
  • Fatigue and Lethargy: New, persistent, or worsening extreme tiredness and lack of energy.
  • Appetite Loss and Unexplained Weight Loss: Experiencing loss of appetite or undergoing unexplained weight loss.

In rarer instances, additional symptoms might include:

  • Finger Clubbing: Distinct changes in finger appearance, such as curvature or enlarged fingertips.
  • Haemoptysis: Coughing up blood.
  • Difficulty Swallowing or Painful Swallowing: Challenges or pain while swallowing.
  • Breathing or Coughing Pain: Aching or discomfort during breathing or coughing.
  • Hoarse Voice: An alteration in voice quality.
  • Face or Neck Swelling: Swelling of the face or neck.
  • Persistent Chest or Shoulder Pain: Continual chest or shoulder discomfort.

Please note that the presence of these symptoms does not definitively indicate lung neuroendocrine tumours, as they can be associated with various other conditions. It’s advisable to consult a medical professional for accurate diagnosis and guidance.

Common symptoms

Less common symptoms


Tests that may be utilised for diagnosing and/or monitoring Lung Neuroendocrine Cancer include:

Blood and/or urine tests:

  • Full blood count
  • Liver and kidney function
  • B12, Iron, Ferritin, Folate levels
  • Chromogranin A
  • Gut hormone profile
  • Calcium
  • Glucose
  • CEA (Carcinoembryonic Antigen)
  • Urinary or serum 5HiAA (serotonin)
  • NT-Pro-BNP (N-terminal pro B-type natriuretic peptide) – for detecting Carcinoid Heart Disease evidence

Clinical evaluation to identify Carcinoid Syndrome and/or other paraneoplastic syndromes like Cushings, SIADH, Hypoglycaemia, and Hypercalcaemia.

In cases of suspected or known MEN1 (Multiple Endocrine Neoplasia type 1): calcium, PTH, and consideration of genetic studies.

Scans & further investigations:

  • Bronchoscopy
  • Endoluminal Bronchoscopic Ultrasound (EBUS)
  • Lung/Respiratory Function Tests
  • Chest x-ray
  • Contrast or High-resolution CT chest
  • CT abdomen and pelvis – to rule out secondary disease and confirm Lung as primary
  • Octreotide (SPECT) or Gallium-Dotatate PET/CT.
  • FDG-PET – when NEC (Neuroendocrine Carcinoma) or rapidly progressing disease is suspected or observed
  • Bone scintigraphy – if bone disease is suspected or present

Echocardiogram – in the presence of Carcinoid Syndrome, elevated Urinary 5HiAA, elevated Pro-NT-BNP, or clinical signs of right-sided heart failure/valve impairment.

Pathology (microscopic examination through specialised tests):

  • Differentiation and cellular morphology
  • Synaptophysin
  • Chromogranin
  • Ki67
  • CD56
  • Cytokeratins
  • TTF1 (Thyroid Transcription Factor 1)

(TTF1, CDX2, Islet1 may aid in distinguishing primary from secondary lung disease).

Please note that these tests serve as diagnostic tools and should be interpreted by medical professionals in conjunction with clinical context.

Diagnosis and grading

Receiving a diagnosis of lung neuroendocrine cancer can be an unexpected and challenging moment in your life. We understand the range of emotions and questions that may arise during this time, and we’re here to provide you with the support and information you need.

Lung neuroendocrine cancer is a complex condition with various subtypes and behaviours. Each diagnosis is unique, and understanding your situation is essential for making informed decisions about your care. It’s important to remember that not all cancers are the same – some may grow slowly, while others are more aggressive. This diversity underscores the significance of understanding your diagnosis and its implications.

Our ‘Just Diagnosed’ page is designed to be your go-to resource during this pivotal phase. We offer valuable information on what to expect, access to specialist care, and emotional support.

More than just information, our website also connects you with a supportive community. Sharing experiences with others who have faced similar challenges can be profoundly comforting and enlightening. We encourage you to explore our online community groups and support groups, where you can find understanding, advice, and a network of individuals ready to uplift you.

Remember, you’re not alone on this pathway. Together, we can navigate the intricacies of lung neuroendocrine cancer and work towards the best possible outcomes. Visit our ‘Just Diagnosed’ page to access vital resources and connect with a community that cares. Your well-being is our priority, and we’re here to stand by your side every step of the way.

Video: Click “Play” to watch Professor Martin Caplin discuss the diagnosis and investigative aspects of Lung NENs.

Understanding Grading

Not all cancers share the same characteristics or exhibit uniform behaviour. Among them, there exist variations in growth speed, invasiveness of nearby structures, and propensity for spreading to other body parts upon diagnosis. While some cancers grow gradually and may not have infiltrated surrounding structures or disseminated, they still possess the potential to do so. On the other hand, certain cancers may have already spread by the time of diagnosis.

The grade of cancer indicates the rate of its growth. You might encounter terms such as ‘Ki67’ or ‘Mitotic Index’ in your clinic letters or medical reports indicating the grade. This classification ranges from Grade 1, representing a slow-growing nature, to Grade 3, signifying a more aggressive and rapidly progressing disease. Understanding the grade provides crucial insights into the behaviour and potential trajectory of the NEN.

How is lung neuroendocrine cancer treated?

When it comes to planning treatments, there are many factors to consider, ensuring that your treatment plan is tailored to your specific needs and the type of NEN you’re dealing with.

It’s important to recognise that your treatment and follow-up strategies could differ significantly even if you share a diagnosis with another patient. The uniqueness of your situation is taken into account for a personalised approach.

Your dedicated care team will discuss your treatment options comprehensively, providing written and verbal information to facilitate an informed decision-making process. Collaboratively, you and your team will choose the most suitable treatment plan. It’s widely recognised that all NEN patients should undergo a specialist Multidisciplinary Team (MDT) assessment.

The available treatment options depend on factors like the type, location, and size of your NEN and whether it has spread and how far. Your overall health, existing health issues, and fitness level are also considered.

When meeting with your medical professionals or specialist nurse/team, the primary objective is to ensure you have all the information necessary to comprehend your condition fully. This empowered understanding is a crucial foundation for your treatment journey.

Video: Click play to watch Professor Martin Caplin talk about Lung NEN treatments – 2020.

Treatment options


Treatment’s primary objective is to ensure optimal care and high quality of life. This involves providing access to suitable treatments, effectively managing symptoms, and addressing your priorities.

The treatment options available are contingent upon factors like the type, location, and size of your cancer and its spread. Additionally, your overall health, existing health conditions, and general fitness level play a role.

One or more of the following approaches might be recommended:

Surgery: Partial or complete removal of the primary neuroendocrine cancer and/or secondary disease sites (metastases).

Control of Disease: Slowing or halting further growth and spread.

Palliation: Easing symptoms you may be experiencing.

Surveillance: Regular monitoring through clinic visits, blood tests, and scans to assess treatment effectiveness or during periods between treatments.

Non-surgical Treatments: These include:

  • Somatostatin Analogues (SSAs): Used to regulate hormone secretion and slow growth in some cases of neuroendocrine cancer.
  • Chemotherapy: Administered orally or intravenously to slow tumour growth or reduce size. Often used in high-grade disease.
  • Targeted Molecular Therapies: Given orally or intravenously to slow tumour growth.
  • Peptide Receptor Radionuclide Therapy (PRRT): Uses targeted radiation to treat neuroendocrine cancer cells.
  • Radiotherapy: Administered post-surgery or as a primary treatment to eliminate remaining cancer cells. Also used for controlling growth in cases of spread, particularly in bone.
  • Endoscopic Procedures: Treatment is provided via procedures like bronchoscopy.
  • Interventional Radiology: Techniques such as embolisation or ablation to treat liver and/or lung-based Neuroendocrine Cancer.
  • Clinical Trials: Essential for developing safe, effective new treatments. Participation is voluntary; discussions with your clinical team can help determine suitability.
  • Symptom Control: A vital aspect of care that occurs throughout the treatment journey. Alleviating pain and managing symptoms contribute to your overall well-being, and strategies range from medication to counselling and practical support.

For more comprehensive information on surgery and other treatments for Lung Neuroendocrine Cancer, please refer to our Lung booklet.

We’re dedicated to ensuring you have the knowledge and resources to make informed decisions about your treatment and care. Our support team is here to assist you with any questions or concerns.

Follow up and management of lung NENs

Select play to watch Professor Was Mansoor’s 2022 talk to the National Lung Natter Group on the management of Lung Neuroendocrine Tumours.

Follow-up protocols are guided by expert-consented guidelines, but practical implementation often varies for valid reasons. Expert-informed and evidence-based guidelines are essential, but equally crucial is tailoring follow-up approaches to suit your individual needs and ensure optimal care.

For Differentiated Neuroendocrine Tumors (DIPNECH):

While no current consensus exists, given its clinical impact and potential for progression, follow-up should be lifelong and driven by clinical presentation.

For Primary Neuroendocrine Cancer of the Lungs:

Typical Carcinoid:

  • After surgery: Review at 3, 6, and 12 months for the first two years, then annually (for life).
  • Imaging: Chest X-ray and bronchoscopy as indicated. CT every three years (or more frequently based on clinical factors). Functional imaging* at one-year post-op and further if recurrence is suspected.

Atypical Carcinoid:

  • Closer monitoring: Review at three months, then every six months for the first five years, and subsequently annually if stable.
  • Imaging: Functional imaging* at one year post-op and if recurrence is suspected. FDG-PET may be preferred in high-grade disease.

Ongoing Therapy:

  • If local progression is suspected, 3-monthly review with potential EBUS.
  • Follow high-grade recommendations for Small Cell Lung Neuroendocrine Carcinoma and Large Cell Neuroendocrine Carcinoma.

High-Grade Follow-up:

  • After surgery: CT every 3-6 months for the first three years, then every 6-12 months.
  • In inoperable or advanced disease: CT every two – three months if on therapy. Repeat biomarkers if elevated at diagnosis.
  • Secondary lung NET: Dictated by lung involvement, respiratory function, and primary site status.

Functional imaging = Octreotide/Gallium-Dotatate and FDG-PET scans.
EBUS = Endoluminal Bronchoscopic Ultrasound Scan.

Advanced Disease:
Follow-up adheres to guidelines but must be informed by prognosis, expected treatment effectiveness, and treatment-related toxicity. Regular reviews should encompass your health, well-being, physical activity, treatment preferences, and the aim of therapy to optimise care planning.

We aim to provide support at every step. Please don’t hesitate to reach out if you have any questions or concerns about your follow-up plan. Your well-being remains our priority.


Causes / risk factors

We do not fully understand the exact causes of NENs; nevertheless, ongoing research is dedicated to unravelling their underlying pathology and biology. Hopefully, these endeavours will ultimately result in a significantly improved comprehension of their origins.

While most NENs do not exhibit a familial tendency, it’s essential to acknowledge the existence of rare conditions that may elevate the risk. Suppose other family members have received a diagnosis of particular cancer, especially at the age of 50 or younger, or have been identified with a cancer-associated genetic condition. In that case, it is essential to communicate this information to your specialist team. Sharing your medical history and insights into your family’s medical background can significantly contribute to comprehensive care.

Recognising the importance of early detection, we encourage regular screenings and check-ups that can aid in identifying NENs at an earlier, potentially more treatable stage. Our commitment extends to providing you with an overview of available treatment options, ranging from surgical interventions to targeted therapies and potential outcomes.

Maintaining a healthy lifestyle plays a crucial role in managing NENs. We advise incorporating a balanced diet, regular exercise, managing stress, and adopting habits that could positively impact your well-being throughout your pathway.


Expert insights

Dive into a wealth of knowledge with our video collection from leading Lung Neuroendocrine Cancer care experts.

Video 2. Click play to watch Dr. Thomas Westwood’s 2022 talk to the National Lung Natter Support Group on “Radiology and Lung Neuroendocrine Tumours“.

Video 1. Click play to watch Professor Was Mansoor’s 2021 talk to the National Lung Natter Support Group on “Things you need to know about Lung Neuroendocrine tumours”.

Stories and experiences

Patient perspective: Twenty years living with lung neuroendocrine cancer

Kate Quirk shares her story of living with lung neuroendocrine cancer and how she is using her experience to help others.

My whole life changed in 2003, with a diagnosis of what was called back then, a Typical Lung Carcinoid.

Living with Neuroendocrine Cancer: Donna's Journey of Strength and Hope

I was diagnosed April 2019 after feeling really unwell for some time and initially being treated for iron deficiency anaemia. I finally got my diagnosis after scans, MRIs etc. and was in total shock.

My Experiences With CAPTEM Chemotherapy and 10 Tips

I was diagnosed with Lung Neuroendocrine cancer in February 2021. This was my second cancer diagnosis in less than five years. It all started with an incidental finding after a surveillance MRI scan in October 2020 following my breast cancer diagnosis and treatment in 2016.

Journey through Diagnosis and Reassurance: Overcoming Challenges with Neuroendocrine Cancer

I was 64 when in February 2020 I was diagnosed with a carcinoid tumour in my left lung. I’d had pneumonia for three months the previous year which began in April 2019, and on one visit to A&E was told I had lung cancer – only to be told three months later that I didn’t have cancer and a few months after that I was discharged from the outpatient clinic.

Unexpected Strength: Embracing Life and Overcoming Neuroendocrine Cancer

November 2019 I was offered a lung screening as part of a so called SUMMIT study at UCL hospital in London. I used to be a smoker and I thought “why not?”. I was feeling very well and very fit. I was certain that all was ok. The day after the CT scan I received a phone call informing me that they had found something in my left lung.

Further support

For those seeking support, we offer resources such as support groups and online groups that provide a platform for patients and their families to connect, share experiences, and find comfort in a strong network.

Additionally, we encourage individuals to consider participating in clinical trials or research studies, as this contributes to the advancement of NEN understanding and treatment and empowers patients to engage in their healthcare journey actively.

Thank you for choosing us as your resource for Lung Neuroendocrine Cancer information. We are dedicated to providing accurate, insightful, and supportive content as you navigate this pathway. If you have any further questions or concerns, please don’t hesitate to reach out.

Natter Support Groups

Our ‘Natter’ Support Groups consist of community-based local, regional, and/or national online gatherings that provide a chance for you, along with your family, friends, and supporters, to connect with others affected by Neuroendocrine Cancer.

NCUK Lung / DIPNECH Support Group

This group operates in conjunction with our Lung Natter Group and welcomes all family and friends of individuals diagnosed with Neuroendocrine Cancer of the Lung and/or DIPNECH.

Lovable Lungoids Facebook Group

This patient-driven group provides resources, information, and support to Lung Neuroendocrine Cancer patients and their supporters.

Download our Lung Handbook

Your personal guide to living with Lung Neuroendocrine Cancer. We aim to provide you with straightforward and valuable information to support you.

Download our Lung Factsheet

Our Neuroendocrine Cancer type specific Factsheets focus on diagnosis, treatment and follow up.

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