Pulmonary Neuroendocrine Tumors

Neuroendocrine tumors (or NETs) start in neuroendocrine cells, which are nerve-like cells that also make hormones. These cells are found all over the body. For adults, neuroendocrine tumors often grow in the digestive tract, but they can also grow in the lungs (lung NETs).

Lung NETs are a rare type of lung cancer. Some lung NETs are not cancerous (benign) while others are cancerous and can spread (metastasize) through the body.

Types & Stages

There are several different types of lung NETs. These groups are defined by how tumor cells look under the microscope. These include:

• Typical carcinoid, a slow-growing type of lung NET that doesn’t typically spread beyond nearby lymph nodes.
   
• Atypical carcinoid, a type of lung NET that can spread to other parts of the body, but typically spreads slower than other types.
   
• Large cell neuroendocrine carcinoma, a more aggressive type of lung NET that can spread to other parts of the body.
   
• Small cell lung cancer, a very aggressive type of lung NET that typically develops in people who have smoked cigarettes for a long time. 

Signs & Symptoms

Symptoms of lung NETs can include: 

• persistent cough
• wheezing
• chest pain
• coughing up blood
• frequent infections in the lungs, such as pneumonia

One in 10 people with lung NETs have tumors that release hormones. If your tumor is releasing hormones, you may experience symptoms like: 

• high blood pressure
• weight gain
• redness and warmth in your face (flushing) 

Diagnosis

At Stony Brook Cancer Center, our doctors use a combination of tests to diagnose lung NETs, such as:

• Chest X-ray, CT, or PET scan, to look inside the lungs.
   
• Biopsy, to take a sample of lung cells and look at them under a microscope.
   
• Bronchoscopy, to look in your lungs. For this test, a thin, flexible tube with a small video camera on the end (a bronchoscope) is inserted down your throat and into your lungs. During the procedure, doctors can remove suspicious tissue to look at it under a microscope (a biopsy).
   
• Blood and/or urine tests, to see if hormones related to lung NETs are in your body.

Treatment

Treatment for lung NETs depends on many factors including where your tumors are growing, how fast they are growing, and whether they are making hormones.

Your treatment may include:

• Watchful waiting, meaning your doctor will closely monitor you but will not start treatment until certain symptoms appear or change. This approach is only used if your lung NET is growing slowly and not causing any major symptoms.
   
• Surgery to remove NETs in the lungs. 
   
• Radiation therapy. At Stony Brook Medicine, most of our radiation treatments are given with x-rays and electron beams generated by machines called linear accelerators.
  Radioactive drugs, or radiopharmaceuticals, which are drugs that deliver radiation directly to cancer cells. A radioactive drug called Lutathera slips into neuroendocrine tumors and releases a radioactive substance that kills the cancer cells. Lutathera treatment requires significant training and a high level of expertise and coordination by a multidisciplinary team. The Stony Brook Cancer Center team is proud to be able to offer this in-demand treatment to patients since 2019.
   
• Hormone therapy, a type of drug that stops your tumors from making extra hormones.
   
• Chemotherapy, a type of drug that stops the growth of cancer cells.
   
• Targeted therapy, which is therapy that is targeted to the genetic or molecular changes in your tumors.

Causes & Risk Factors

There are only a few things that are known to raise the risk of developing lung neuroendocrine tumors.

Smoking tobacco is known to raise the risk of developing a type of lung NET called small cell lung cancer (SCLC). Almost every person who is diagnosed with SCLC (95 percent of people) has a history of smoking.

If you are born with a genetic disorder called MEN1 syndrome, your risk of developing a lung NET is higher than someone who does not have this syndrome.

Screening

Screening for lung cancer is done with a test called low dose CT, or LDCT.

Experts recommend getting screened for lung cancer only if you are at high risk of developing the disease. You are considered to be at high risk of developing lung cancer if you: 

• are between 50 and 80 years old,
• have a 20 or more pack-year smoking history,
• and you still smoke or have quit smoking within the last 15 years.

If you are considered to be at high risk, you should get screened with LDCT once a year.