Pulmonary Carcinoid Tumors

Pulmonary carcinoid tumors are rare tumors that grow in the lungs. These tumors grow from neuroendocrine cells, nerve-like cells that also make hormones. Neuroendocrine cells are found all over the body, including the lungs and the digestive tract. Both children and adults can develop a pulmonary carcinoid tumor.

Types & Stages

There are two types of pulmonary carcinoid tumors. They are defined by how tumor cells look under the microscope. They are:

• Typical carcinoid, a slow-growing tumor that doesn’t typically spread (metastasize) to other parts of the body. This is the most common type of lung carcinoid tumor.
   
• Atypical carcinoid, a tumor that tends to grow faster than typical carcinoid tumors, and can spread (metastasize) to other parts of the body.

If you are diagnosed with a pulmonary carcinoid tumor, your doctor will run tests to find out what stage it is. The stage means how big the tumor or tumors are and whether they have spread outside of the lungs. Knowing the stage will help your doctor figure out the best treatment for you.

Signs & Symptoms

Symptoms of pulmonary carcinoid tumors include:

• cough
• shortness of breath
• wheezing
• chest pain

Some carcinoid tumors make hormones. In rare cases, a tumor may make enough hormones to cause symptoms like:

• weight gain
• redness and warmth in your face (flushing)
• severe diarrhea
• fast heartbeat

However, some people with pulmonary carcinoid tumors don’t have any noticeable symptoms.

Diagnosis

At Stony Brook Cancer Center, our doctors use a combination of tests to diagnose pulmonary carcinoid tumors, 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 carcinoid tumors are in your body.

Because many pulmonary carcinoid tumors don’t cause noticeable symptoms, many are found accidentally during imaging tests being done for other reasons. 

Treatment

Treatment for pulmonary carcinoid tumors 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:

• Surgery to remove tumors in the lungs and nearby lymph nodes. Sometimes only a small piece of the lung needs to be removed. Other times, a whole lobe or one whole lung is removed. 
   
• 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.
   
• 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.
   
• 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.

At Stony Brook Medicine, our Palliative Care Service is available to everyone, no matter the stage of your cancer. This specialized team helps patients and their families cope with the physical, emotional, and spiritual distress of cancer. They can provide relief from symptoms of pulmonary carcinoid tumor and/or its treatment, such as pain, nausea, fatigue, and loss of appetite.

Causes & Risk Factors

There is very little known about what raises your risk of developing a pulmonary carcinoid tumor.

The factors that are known to raise the risk of pulmonary carcinoid tumors are:

• Having MEN1 syndrome, a genetic disorder that you are born with. Around 1 in 10 people with MEN1 syndrome will develop a carcinoid tumor.
• Smoking tobacco, which may raise the risk of developing an atypical carcinoid tumor.

Screening

There is no recommended screening test for pulmonary carcinoid tumors. If you have MEN1 syndrome, your doctors may recommend yearly screening with blood and/or imaging tests to check for carcinoid and other tumors.