Neuroendocrine tumors (patient information)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2] Ujjwal Rastogi, M.B.B.S. [3]

Overview[edit | edit source]

Neuroendocrine tumors (NETs) are cancer that arise from cells of the endocrine (hormonal) and nervous systems. Many are benign, while some are malignant. They most commonly occur in the intestine, but are also found in the lung and the rest of the body. A pancreatic islet cell tumor is a rare tumor of the pancreas that starts from a type of cell called the islet cell.

What are the symptoms of Neuroendocrine tumors?[edit | edit source]

Symptoms depend on which hormone is made by the tumor.

• For example, insulinomas produce insulin, which helps the body lower blood sugar levels. Symptoms may include:
  • Feeling tired or weak
  • Shaking or sweating
  • Headache
  • Hunger
  • Nervousness, anxiety, or feeling irritable
  • Unclear thinking or feeling uneasy
  • Double or blurry vision
  • Fast or pounding heartbeat

If your blood sugar gets too low, you may faint, have a seizure, or even go into a coma.

• Gastrinomas make the hormone gastrin, which tells the body to make stomach acid. Symptoms may include:
  • Abdominal pain
  • Diarrhea
  • Ulcers in the stomach and small bowel
  • Vomiting blood (occasionally)

• Glucagonomas make the hormone glucagon, which helps the body raise blood sugar levels. Symptoms can include:
  • Diabetes
  • Red, blistery rash in the groin or buttocks
  • Weight loss

What causes Neuroendocrine tumors?[edit | edit source]

In the normal pancreas, cells called islet cells produce hormones that regulate a variety of bodily functions, such as blood sugar level and the production of stomach acid.

Tumors that arise from islet cells of the pancreas can also produce a variety of hormones, though some do not.

Although islet cells produce many different hormones, most tumors release only one hormone that leads to specific symptoms. Pancreatic islet cell tumors can be noncancerous (benign) or cancerous (malignant).

Islet cell tumors include:

• Gastrinomas (Zollinger-Ellison syndrome)
• Glucagonomas
• Insulinomas

Who is at highest risk?[edit | edit source]

A family history of multiple endocrine neoplasia, type I (MEN I) is a risk factor for islet cell tumors.

Diagnosis[edit | edit source]

• Blood tests may vary depending upon the symptoms, but may include:
  • Fasting glucose level
  • Gastrin level
  • Glucose tolerance test
  • Secretin stimulation test for pancreas
  • Blood glucagon level
  • Blood insulin C-peptide
  • Blood insulin level
• The following imaging tests may be performed:
  • Abdominal CT scan
  • Abdominal ultrasound
  • Endoscopic ultrasound
  • MRI of abdomen

• A blood sample may also be taken from a vein in the pancreas for testing.

• Sometimes, surgery is needed to diagnose and treat this condition. During this procedure, the surgeon examines the pancreas by hand and with ultrasound.

When to seek urgent medical care?[edit | edit source]

Call your health care provider if you develop symptoms of these tumors, especially if you have a family history of MEN I.

Treatment options[edit | edit source]

Treatment depends on the type of tumor and if it is cancerous.

• Cancerous tumors can grow quickly, and spread to other organs. They may not be treatable. Tumors are usually removed with surgery, if possible.
• If cancer cells spread to the liver, a part of the liver may also be removed, if possible. If the cancer is widespread, chemotherapy may be used to try and shrink the tumors.
• If the abnormal production of hormones is causing symptoms, you may receive medications to counteract their effects. For example, with gastrinomas, the overproduction of gastrin leads to too much acid in the stomach. Medications that block acid release can reduce symptoms.

Where to find medical care for Neuroendocrine tumors?[edit | edit source]

Directions to Hospitals Treating Neuroendocrine tumors

Prevention of Neuroendocrine tumors[edit | edit source]

There is no known prevention for these tumors.

What to expect (Outlook/Prognosis)?[edit | edit source]

You may be cured if the tumors are surgically removed before they have spread to other organs. If tumors are cancerous, chemotherapy may be used, but it usually cannot cure patients.

Life-threatening problems (such as very low blood sugar) can occur due to excess hormone production, or if the cancer spreads throughout the body.

Possible complications[edit | edit source]

• Diabetes
• Hormone crises (if the tumor releases certain types of hormones)
• Severe low blood sugar (from insulinomas)
• Severe ulcers in the stomach and small intestine (from gastrinomas)
• Spread of the tumor to the liver

Source[edit | edit source]

National Library of Medicine