Neuroendocrine Tumors

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Neuroendocrine Tumors (Carcinoid Tumors) in Children

Neuroendocrine Carcinoid Tumors in Children Related Articles

What Are Neuroendocrine (Carcinoid) Tumors?

What is the pancreas?
The pancreas is an organ that is part of the digestive system. It sits behind and below the stomach in the back of the abdominal cavity. Neuroendocrine tumors form in the pancreas.

Neuroendocrine tumors (including carcinoid tumors) usually form in the lining of the stomach or intestines, but they can form in other organs, such as the pancreas, lungs, or liver. These tumors are usually small, slow-growing, and benign (not cancer).

  • Some neuroendocrine tumors are malignant (cancer) and spread to other places in the body.
  • Sometimes neuroendocrine tumors in children form in the appendix (a pouch that sticks out from the first part of the large intestine near the end of the small intestine).
  • The tumor is often found during surgery to remove the appendix.

What Are the Signs and Symptoms of Neuroendocrine (Carinoid) Tumors in Children?

Some neuroendocrine tumors release hormones and other substances. If the tumor is in the liver, high amounts of these hormones may remain in the body and cause a group of signs and symptoms called carcinoid syndrome. Carcinoid syndrome caused by the hormone somatostatin may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
  • Redness and a warm feeling in the face and neck.
  • A fast heartbeat.
  • Trouble breathing.
  • Sudden drop in blood pressure
    • (restlessness,
    • confusion,
    • weakness,
    • dizziness, and
    • pale, cool, and clammy skin).
  • Diarrhea.
Other conditions that are not neuroendocrine tumors may cause these same signs and symptoms.

How Are Neuroendocrine (Carcinoid) Tumors Diagnosed?

Tests that check for signs of cancer are used to diagnose and stage neuroendocrine tumors. They may include:

  • Physical exam and history.
  • Blood chemistry studies.

Other tests used to diagnose neuroendocrine tumors include the following:

  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
    • The number of red blood cells, white blood cells, and platelets.
    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
    • The portion of the blood sample made up of red blood cells.
  • Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances, such as hormones. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The urine sample is checked to see if it contains a hormone made by carcinoid tumors. This test is used to help diagnose carcinoid syndrome.
  • Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find tumors. A very small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.

What Is the Treatment and Prognosis for Neuroendocrine (Carcinoid) Tumors in Children?

Treatment of neuroendocrine tumors in the appendix in children may include the following:

  • Surgery to remove the appendix, when the tumor is small and only in the appendix.
  • Surgery to remove the appendix, lymph nodes, and part of the large intestine, when the tumor is larger, has spread to nearby lymph nodes, and is in the appendix.

Treatment of neuroendocrine tumors that have spread to the large intestine, pancreas, or stomach is the same as treatment for adult high-grade neuroendocrine tumors.

Treatment of recurrent neuroendocrine tumors in children may include the following:

  • A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.

The prognosis for neuroendocrine tumors in the appendix in children is usually excellent after surgery to remove the tumor. Neuroendocrine tumors that are not in the appendix are usually larger or have spread to other parts of the body at the time of diagnosis and do not respond well to chemotherapy. Larger tumors are more likely to recur (come back).


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Last updated Oct. 6, 2017