Lung Neuroendocrine Tumors (NETs)

Lung NETs are rare and complex—this guide walks you through their types, symptoms, and treatment options so you can better understand what to expect.

What Are Lung NETs?

Lung neuroendocrine tumors (NETs) are rare tumors that form from neuroendocrine cells within the lungs. Neuroendocrine cells can be found in other organs throughout the body, but lung NETs come from the ones located inside the lungs. They can grow slowly or quickly depending on the type. There are four main types of lung NETs (Typical Carcinoid, Atypical Carcinoid, Large Cell Neuroendocrine Carcinoma, and Small Cell Lung Carcinoma). Types of lung NETs are grouped by how the cells look under the microscope and how fast they grow. They're different from other lung cancers, may not cause symptoms early on, and require personalized diagnosis, treatment, and long-term monitoring.

Quick Facts:

  • Lung NETs are rare, making up only 1–2% of all lung cancers.
  • NETs develop more often in the digestive tract than in the lungs. Only about 1 out of 5 NETs start in the lungs.
  • They can be noncancerous or cancerous and grow at different speeds.
  • Symptoms may not appear until later stages.
  • Surgery is a common treatment, though options vary based on the tumor type and stage.

How Lung NETs Form

Your lungs contain many different types of cells. These cells have different jobs like controlling breathing or keeping germs out. One type of cell in your lungs (and throughout your body) is called a neuroendocrine cell. These cells are similar to both nerve cells and hormone-producing cells. In the lungs, these cells respond to signals from the body to help with lung function.  

Sometimes these cells can grow uncontrollably and turn into a tumor in the lungs called a neuroendocrine tumor (NET). Not all NETs are cancerous. Cancerous NETs are sometimes called neuroendocrine neoplasms or neuroendocrine carcinomas. Because NET cells are throughout the body, this can happen places other than the lung. The digestive tract is the most frequent location for NETs, while the lung is the second most common.  

Learn how lung NETs are different from non-small cell or small cell lung cancer.
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What Causes Lung NETs? 

Researchers are still trying to understand what causes NETs in the lungs. In some cases, tobacco use may increase your risk of developing lung NETs. Another risk factor may be a rare genetic disorder called Multiple Endocrine Neoplasia Type 1 (MEN1). In most cases, it is unclear what has caused a person to develop NETs.

A rare non-cancerous condition called DIPNECH (Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia) can turn into NETs. Treatment options for DIPNECH vary based on the type of NET and its characteristics. 

Lung NETs often affect people in their 60s, and they are slightly more common in females. 

Types of Lung NETS 

There are four different types of lung NETs. They are named for how the cells look under the microscope. Below is an overview of the subtypes of lung NETs. 

Type of NETLocationGrade (how quickly the cells are dividing)Growth SpeedDoes it typically spread?Associated with Tobacco Use

Typical Carcinoid (TC)

The center of the chest (central)

1 (Low)

Slow

No

No

Atypical Carcinoid (AC)

Towards the edge of the lung (peripheral)

2 (Intermediate)

Slightly faster, but still slow

A little more likely to spread than TC

No

Large Cell Neuroendocrine Carcinoma (LCNEC)

Can be central or peripheral

3 (High)

Fast growing

Likely to spread

Yes

A form of small cell lung cancer (SCLC)

Originates in the central bronchi (large tubes that carry air from your windpipe to your lungs) but often spreads

3 (High)

Fastest growing

Likely to spread

Yes

Learn more about the characteristics of lung NETs and how they impact treatment. 

Symptoms of Lung NETs

Symptoms are often related to the characteristics of the tumor(s) like size, location and functionality (whether the tumor is releasing hormones into the bloodstream). Some NETs do not cause any symptoms until they are at a later stage, which makes early detection and treatment more challenging.

Respiratory (breathing) symptoms are the most common among patients with lung NETs. Symptoms often include: 

  • Cough
  • Shortness of breath
  • Wheezing
  • Coughing up blood

Lung NETs can either be functional or non-functional, with the majority of lung NETs being nonfunctional. A functional tumor can overproduce hormones and amino acids and leak them into the bloodstream (a nonfunctional tumor does not). Functional tumors can cause a range of symptoms such as flushing (face redness), diarrhea and wheezing. This collection of symptoms is sometimes called carcinoid syndrome. A functional NET can also cause a related hormonal syndrome called Cushing’s syndrome. 

Symptoms like unexplained weight loss, fatigue and fever may appear, especially with the more advanced stages of NETs.

You may develop symptoms based on where the cancer has spread (metastisized). This could include symptoms like pain in other parts of the body as well as neurological symptoms (headache, vision changes, confusion) if the cancer has spread to the brain. 

Be sure to speak with your physician if you are experiencing any concerning symptoms. 

Diagnosis of Lung NETs

Lung NETs are usually found on a CT scan, either after a person has breathing symptoms or “incidentally” (when looking for something else). The CT scan is followed by a biopsy, where physicians remove a sample from the tumor to confirm the makeup of the tumor. You may receive other procedures and blood tests to learn more about your exact cancer.

Treatment of Lung NETs 

Treatment for lung NETs is based on the unique characteristics of your tumor. This includes the type, grade, stage and location. Treatment also depends on your overall health and any other health conditions you may have. It is important to work closely with a physician who is up to date on the treatment of lung NETs, as there are new and emerging treatments being studied. In some cases, a physician might recommend “watchful waiting” if the tumor appears slow growing. Below is an overview of the types of treatment typically used to treat lung NETs. Not every treatment option is the right choice for every patient. Your physician can help you understand your options and what to expect. 

Surgery to remove the tumor(s) and surrounding lymph nodes is often considered for patients who have NETs, especially typical and atypical carcinoids. Some LNEC patients may receive surgery if they are candidates. SCLC patients usually do not receive surgery because it is likely the tumor has spread by the time it is diagnosed. 

How does a patient know if they are a candidate for surgery? 

Your physician considers the characteristics of your tumor like size, location and make-up to see if it can be safely removed through surgery. Your physician will also consider your overall health and if surgery is the right choice for you. Typically, surgery to remove the tumor is an option for earlier stage patients versus later stage.  

What type of lung surgery is recommended for patients with lung NETs? 

There are many different surgical techniques that physicians can use to remove the tumor and sometimes surrounding lymph nodes or tissue. One common surgical procedure used in patients with lung NETs is called a wedge resection which removes a section from the lung. Many surgeries are done using minimally invasive techniques which allow the surgeon to perform the surgery with small incisions. The main goal of surgery for lung NETs is to completely remove (resect) cancerous tissue and have negative or clear margins (no cancer cells in the edges of the tissue that was removed).  

Is surgery typically an option if the cancer has spread outside the lung? 

Typically surgery is not used to treat lung cancer that has spread throughout the body (but may be an option if the cancer has spread locally, or just around the lung). But, because lung NETs commonly spread to the liver, some patients may be candidates for liver surgery. Removing tumors from the liver may help with uncomfortable symptoms and may improve prognosis. 

Radiation therapy (RT) uses strong beams of energy or small radioactive molecules to kill cancer cells. There are different types of RT, including a type of nuclear medicine called Peptide Receptor Radionuclide Therapy (PRRT). PRRT binds to the tumor cells and destroys them with a small, but powerful dose of radioactivity. For patients with lung NETs, RT can have several uses.

  • RT is sometimes given before or after surgery to either shrink the tumor or kill remaining cancer cells.
  • Patients who are not candidates for surgery or have positive margins (cancer cells remaining near where the tumor was) may receive RT (with or without chemotherapy) to shrink the tumor or kill remaining cancer cells.
  • RT can also help with symptom relief in the lungs and places the cancer has spread, like in the bones. 

Chemotherapy is mostly used to treat the LCNEC and SCLC subtypes of lung NETs. Treatment usually includes two different types of chemotherapy given at the same time. A platinum-based chemotherapy (like carboplatin or cisplatin) and a slightly different type of chemotherapy medication (options commonly include etoposide or irinotecan). 

Sometimes chemotherapy (with or without radiation) is used before or after lung NETs surgery. 

Much research is being done to understand the role of targeted therapy in treating lung NETs. Targeted therapies work by blocking exactly what is causing the tumor to grow. 

Some targeted therapies used to treat lung NETs may include:

  • Everolimus (a type of targeted therapy called an mTOR inhibitor that blocks the mTOR growth pathway)
  • Cabozantinib (a type of targeted therapy called a tyrosine kinase inhibitor, or TKI)
  • Somatostatin analogs (SSAs) (medications used to regulate hormone production) like octreotide and lanreotide
  • In rare cases some lung NETs will have mutations that typically appear in non-small cell lung cancer and can be treated with an approved targeted therapy.

Immunotherapy (IO) harnesses the power of the immune system to fight cancer. For lung NETs, IO is typically only used to treat SCLC. Learn more about immunotherapy

For people facing any type of cancer, a clinical trial might be the best treatment option available. Clinical trials are highly monitored research studies and may provide access to emerging treatments for lung NETs. Talk with your doctor to find out if you are eligible for a clinical trial. 

There are several procedures that aim to shrink or remove a NET tumor without surgery. These procedures usually do not provide a cure, but they may improve lung NET symptoms or slow tumor growth. Procedures that help improve symptoms and quality of life are often part of supportive, or palliative care. 

Ablation

Ablation treatments use extreme heat or cold to destroy tumors. Common ablative procedures are radiofrequency ablation (RFA) and microwave thermotherapy.

Embolization

Embolization treatments use substances injected into an artery to try to block the blood flow to cancer cells. The three main types of embolization are arterial embolization, chemoembolization and radioembolization.

Lung NET patients may experience a build up of fluid around the lungs (pulmonary edema). Thoracentesis, pleurodesis and catheter placement are three procedures used to drain fluid around the lung. 

Living with Lung NETs

Monitoring Lung NETs 

It is important to monitor most patients with lung NETs for disease growth or spread. Patients who are not candidates for surgery will receive scans as needed so physicians can monitor how well the cancer is responding to treatment. 

Patients with earlier stage disease who get surgery (with the intent to cure the disease) may receive chest and abdomen CT scans every six months for the first two years and then yearly for at least 10 years. Low-grade (typical) lung NETs that do not involve the lymph nodes are unlikely to come back, so those patients are unlikely to receive regular scans after surgery to look for new nodules. Talk with your physician about what you can expect during and after treatment.

Challenges for Patients with Lung NETs

Patients with lung NETs face some unique challenges. Lung NETs account for only 1 to 2 percent of all cancers in the lung. Because lung NETs can be rare, researchers are still working to try and find the best way to detect, treat and follow-up with these cancers. As researchers work to identify best practices, there can be confusion and discrepancies about how to best treat lung NETs. It is important to work with a care team who are up to date on the latest research. 

Another challenge is misdiagnosis. The symptoms of lung NETs can be similar to a variety of other diseases which causes delayed and mis-diagnoses.  

Patients with lung NETs may also struggle with stigma and isolation. Lung cancer resources are usually focused on non-small and small-cell lung cancer and patients with lung NETs may feel excluded from support offerings, or may find it challenging to find other patients in their shoes. Also, because of lung cancer’s connection to tobacco use, it is not uncommon for some patients to feel embarrassed or ashamed if they used tobacco. Patients may feel frustrated and stigmatized when they share they have a cancer of the lungs, as much of the public’s first question after hearing about a lung cancer diagnosis is “Did you smoke?”

It is important to work closely with your physicians to help monitor your medication side effects. Ask about connecting with a supportive/palliative care doctor at the beginning of your treatment to help ensure your side effects are well managed.

Lung cancer research can move at a rapid pace. Always speak with your doctor about the most up-to-date treatment guidelines.

Page last updated: May 20, 2026

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