Lung Carcinoid Tumors

What are lung carcinoid tumors?

A lung carcinoid tumor is a type of cancerous tumor that starts in the neuroendocrine cells in your lungs. Neuroendocrine cells help regulate many of your body’s functions, like moving food through your digestive tract and air through your lungs.

Typical lung carcinoid tumors — the most common kind — grow slowly and rarely spread (metastasize) to other parts of your body. Atypical lung carcinoid tumors tend to grow faster and are more likely to spread to other organs.

Carcinoid tumors can start in many parts of your body, including your digestive tract, appendix, kidneys and other organs. About 1 in 3 start in your lungs.

Typical carcinoid tumors

Typical carcinoid tumors account for up to 90% of lung carcinoid tumors. They’re slow-growing and don’t often metastasize elsewhere in your body. Typical carcinoid tumors can be central carcinoids (located in the large airways in your lungs) or peripheral carcinoids (found around the edges of your lungs and small airways).

Atypical carcinoid tumors

Atypical lung carcinoid tumors are much less common than typical lung carcinoids and tend to grow faster. They have a greater chance of spreading to other organs. They’re also more likely to be associated with smoking than typical carcinoids. Atypical lung carcinoid tumors are more often peripheral carcinoids, but they’re sometimes found in your large airways.

How common are lung carcinoid tumors?

Lung carcinoid tumors are rare. About 2,000 to 4,500 new cases of lung carcinoid tumors are diagnosed in the U. S. each year. Only 1% to 2% of all lung cancers are carcinoid tumors.

What are the symptoms of lung carcinoid tumors?

Symptoms of lung carcinoid tumors include:

• Cough, sometimes with blood or mucus.
• Chest pain.
• Shortness of breath (dyspnea).
• Wheezing.

If the tumor is creating a lot of the hormone serotonin, you might have symptoms of carcinoid syndrome, including:

• Severe diarrhea.
• Redness or warmth (flushing) in your face and neck.
• Unexpected weight gain.
• Wheezing, shortness of breath or asthma-like symptoms.

Many people with lung carcinoid tumors don’t have symptoms.

What causes lung carcinoid tumors?

When neuroendocrine cells make too many copies of themselves, they can create tumors. Depending on how they behave and look under a microscope, they might be classified as carcinoid tumors, large cell neuroendocrine carcinomas or small cell lung cancer. We don’t know why these cells start growing out of control and create lung tumors.

Neuroendocrine cells are found in many organs in your body. They act like both nerve cells (they receive signals from your nervous system) and endocrine cells (they respond to those signals by releasing hormones). Neuroendocrine cells don’t form organs, but help regulate some of your lungs’ functions, like:

• Airflow.
• Blood flow in your lungs.
• Lung cell growth.
• Adjustments to different levels of oxygen and carbon dioxide in the air.

What are the risk factors for lung carcinoid tumors?

While there’s no clear cause of lung carcinoid tumors, some people may be at a higher risk. You might be at a higher risk if you:

• Have multiple endocrine neoplasia type 1 (MEN1), a rare inherited disorder.
• Smoke.
• Are female.
• Are between 45 and 55 years old. This is the most common age at diagnosis, but people of any age, even kids, can develop a carcinoid tumor.

What are the complications of lung carcinoid tumors?

Complications of lung carcinoid tumors include:

• Pneumonia.
• Blockage in your lungs.
• Carcinoid syndrome.

How are lung carcinoid tumors diagnosed?

Diagnosing tumors can be a long process with many steps. When you first visit a provider, they’ll ask you about your symptoms and listen to your heart and lungs.

If they think a lung issue is causing your symptoms, they’ll get imaging (like a chest X-ray) to look for changes. If the pictures of your lungs show a tumor, you’ll need to have blood tests and a biopsy to confirm the type and guide treatment.

What tests will be done?

Some tests you may need include:

• Imaging. A provider looks for evidence of lung tumors on chest X-rays or CT scans (computed tomography scans).
• Blood and urine tests. Providers can use blood or urine (pee) tests to check for elevated amounts of certain hormones that neuroendocrine tumors make.
• Biopsy. Your provider may get a sample of the tumor to examine under a microscope. A common way to get this sample is with bronchoscopy.

How are lung carcinoid tumors treated?

Your treatment plan will depend on the size of the tumor, its location and your overall health. Many lung carcinoid tumors can be treated with surgery alone. If cancer has spread to other organs or if the entire tumor can’t be removed, you may need additional treatments.

What are the treatment options for lung carcinoid tumors?

Your treatment options may include:

• Surgery. A surgeon may remove part of your lung with a lobectomy or wedge resection. In rare cases, they might remove your entire lung (pneumonectomy). Sometimes, surgeons also remove tumors that have spread to other organs in your body.
• Lymph node removal. A surgeon may remove nearby lymph nodes to determine if the tumor has spread to these nodes and to reduce the risk of the tumor spreading to other parts of your body.
• Radiation therapy. Providers usually use external-beam radiation to treat carcinoid tumors. They also sometimes use brachytherapy (internal radiation) where they place pellets or rods of radiation close to the tumor.
• Octreotide (Sandostatin®). Providers can use this medication — a type of hormone — to treat carcinoid syndrome.
• MTOR inhibitors. Medications like everolimus (Afinitor®) can help prevent tumors from growing.
• Chemotherapy. Providers don’t typically use traditional cancer chemotherapy to treat carcinoid tumors since they’re almost always slow-growing (and chemotherapy works on fast-growing cells). But you might need chemotherapy if you have a fast-growing cancer.

Can you prevent lung carcinoid tumors?

There’s no specific way to prevent lung carcinoid tumors. But if you smoke, quitting can reduce your risk of many kinds of lung diseases. Ask your provider for helpful resources.

Are lung carcinoid tumors curable?

It can be hard to say if cancer is completely gone, or cured, after treatment — there’s always a chance it can come back (recur). But typical lung carcinoid tumors are very treatable. The likelihood of a typical carcinoid tumor recurring is very low — less than 4%.

Atypical lung carcinoid tumors are harder to treat. Your provider may recommend a few different types of treatments. About 1 in 3 people with atypical carcinoid lung tumors experience recurrence within four years of treatment.

What is the survival rate for carcinoid lung cancer?

The overall five-year relative survival rate for all types of carcinoid lung tumors (including those that have spread outside of your lungs) is 89%. The prognosis (outlook) for typical lung carcinoid tumors is better than for atypical tumors.

How do I take care of myself?

Follow your care team’s recommendations on the best way to take care of yourself after any surgeries or other treatments. Attend all your follow-up visits with your providers. They’ll monitor you for complications or recurrences.

When should I see my healthcare provider?

Let your providers know if you experience any new or worsening symptoms, like difficulty breathing, diarrhea or unexpected weight loss. Don’t hesitate to contact your provider if you have any questions or concerns.

When should I go to the ER?

Go to the emergency room right away if you have severe symptoms, including:

• Struggling to breathe or getting short of breath while sitting still.
• Coughing up blood.
• New or worsening chest pain.
• Confusion or altered mental state.
• Blue skin, nails or lips (cyanosis).

What questions should I ask my doctor?

It might be helpful to ask your provider:

• Is this a typical or atypical carcinoid tumor?
• Has it spread?
• What treatment do you recommend?
• What’s the outlook for this type of tumor?
• How can I manage symptoms at home?