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MyPART - My Pediatric and Adult Rare Tumor Network
 

Carcinoid Tumor

What is carcinoid tumor? 

Carcinoid tumor is a type of neuroendocrine tumor that grows from neuroendocrine cells. Neuroendocrine cells receive and send messages through hormones to help the body function. Neuroendocrine cells are found in organs throughout the body.

Carcinoid tumors often grow very slowly. In children and young adults, carcinoid tumors are most often found in the appendix, called appendiceal carcinoid tumors, or in the lungs, called bronchial tumors. In adults, carcinoid tumors are most often found in the digestive tract. This tumor may spread to other parts of the body but does so more often in adults than children. 

How common is carcinoid tumor?

Carcinoid tumor is rare in children and more common in adults. Experts think that carcinoid tumor affects 4 in 100,000 adults. Carcinoid tumor in children and young adults is so rare that there is little data on how many young people have it.

How is carcinoid tumor diagnosed?

Some people with carcinoid tumors have symptoms, but others don’t. The symptoms of carcinoid tumor depend on where the tumor is inside the body. 

Patients with carcinoid tumor of the appendix usually have symptoms of appendicitis, such as pain in the abdomen. They may be diagnosed later with carcinoid tumor if the doctor removes the appendix and finds a tumor. Patients with carcinoid tumor in other parts of the digestive tract may have symptoms such as:

  • Pain in the abdomen
  • Nausea or vomiting
  • Diarrhea

Patients with carcinoid tumor in the lungs may have symptoms such as:

  • Trouble breathing
  • Chest pain
  • Wheezing 
  • Coughing up blood

Sometimes these symptoms are diagnosed as pneumonia by mistake. 

In rare cases, patients with carcinoid tumor may develop carcinoid syndrome. Carcinoid syndrome is a problem that develops from the tumors making hormones.  Symptoms include:

  • Feeling flushed
  • Nausea and vomiting
  • Diarrhea 

Lab Tests: If you have symptoms of carcinoid tumor, your doctor will order lab tests of your urine or blood to check your hormone levels.

Imaging: Your doctor will use scans such as CT and MRI to see where the tumor is and how big it is. Different types of PET scans can also help find more fast-growing neuroendocrine cancer cells. 

Biopsy: To check if the tumor is carcinoid tumor your doctor will do a biopsy, taking a small sample from the tumor with a needle. An expert, called a pathologist, will study cells from the sample under the microscope and run other tests to see what kind of tumor it is. 

How is carcinoid tumor treated?

Treatment for each person will be unique. You should go to an expert in neuroendocrine tumor treatment to decide the best approach for your tumor. You can contact MyPART for help finding experts near you.

Surgery: If you have a carcinoid tumor, you may have surgery to remove the tumor and some surrounding tissue. Surgery is the best option for treating carcinoid tumor and preventing it from spreading.

When the carcinoid tumor is large or the cancer cells have spread to other parts of the body other treatments may include:

Somatostatin analogs: Somatostatin analogs are a type of treatment that may stop your body from making too many hormones.  This may slow down the growth of the tumor when cancer cells have spread to other part of the body

Targeted therapy: Targeted therapy is a type of treatment that uses drugs that target certain genes or proteins to kill cancer cells. Neuroendocrine tumor cells have receptors on the surface of the cells called somatostatin. A type of targeted therapy called peptide receptor radionuclide therapy (PRRT) can target these cells.

Chemotherapy: is a type of treatment that uses stronger drugs to kill fasting growing cells. 

Does carcinoid tumor run in families?

Carcinoid tumor does not seem to run in families. But people with a genetic condition that can run in families called multiple endocrine neoplasia type 1 (MEN1) do have a higher risk of getting carcinoid tumor.

How does carcinoid tumor form?

We do not know what causes carcinoid tumor to form. Scientists are always working to understand how cancer starts, but it can be hard to prove. We know that patients with a condition called multiple endocrine neoplasia type 1 (MEN1) with changes in the gene called MEN1 have a higher chance of developing bronchial and intestinal carcinoid tumor. So this gene may play a role in carcinoid tumors of the lung and digestive tract. 

What is the prognosis for someone with carcinoid tumor?

The estimate of how a disease will affect you long-term is called prognosis. Each person is different and prognosis will depend on many factors, such as: 

  • Where the tumor is in your body
  • If the cancer has spread to other parts of your body
  • How much of the tumor was taken out during surgery 

If you want information on your prognosis, it is important to talk to your doctor. Also, NCI has resources to help you understand cancer prognosis

Doctors estimate carcinoid tumor survival rates by how groups of people with carcinoid tumor have done in the past. In children, because there are so few cases of carcinoid tumor, these rates may not be very accurate. 

The prognosis for children and young adults who have surgery to remove the tumor have a very good prognosis. Some studies show the 5-year survival rate for children and young adults with bronchial carcinoid tumor that has been removed is over 90%. Prognosis for people whose carcinoid tumor has spread to other parts of the body may be lower.