Sarah Cannon - February 01, 2023

Neuroendocrine cancer—also known as a neuroendocrine tumor (NET)—is a disease that affects certain cells in the neuroendocrine system. Neuroendocrine cells receive signals from the nervous system and then release hormones into the bloodstream in response to these signals. The cells are present in the body’s organs and help regulate bodily functions. Neuroendocrine cells have similar traits to both nerve cells and hormone-producing endocrine cells.

Neuroendocrine tumors typically grow slowly, though some are more aggressive. They can occur anywhere in the body, though they are most common in the:

  • Lungs and bronchial system
  • Large intestine
  • Small intestine
  • Appendix
  • Pancreas

In about 15% of cases, the primary tumor site (where the cancer cells began) is unknown.

How common is neuroendocrine cancer?

Neuroendocrine cancer affects approximately 175,000 people in the United States, making it a relatively rare cancer.

Neuroendocrine cancer symptoms

In its early stages, neuroendocrine cancer rarely causes symptoms. A tumor may be discovered during an unrelated surgery or imaging test.

Symptoms of neuroendocrine cancer can include:

  • Unexplained weight loss
  • Loss of appetite
  • Fatigue
  • A thickening or lump in the body
  • Nausea
  • Vomiting
  • Persistent pain in a certain area of the body
  • Chronic cough or hoarseness
  • Unusual discharge or bleeding
  • Changes in bladder or bowel habits
  • Diarrhea
  • Facial redness
  • Skin rash
  • Ulcer disease
  • High blood glucose (hyperglycemia)
  • Low blood glucose (hypoglycemia)
  • Jaundice (yellowing of the whites of the eyes and skin)
  • Anxiety
  • Confusion
  • Nutritional deficiencies
  • Wheezing
  • Weakness
  • Fast heartbeat
  • Sweating
  • High blood pressure
  • Scarring of the heart valves (carcinoid heart disease)

Intense exercise, drinking alcohol, and stress can worsen some of these symptoms. Additionally, certain foods may trigger symptoms, including foods high in:

  • Serotonin, such as tomatoes, walnuts, bananas, pecans, and plantains
  • Amines, such as smoked meat and fish, tofu, sauerkraut, aged cheeses, and yeast extracts

These symptoms can also be a sign of other health conditions, not necessarily neuroendocrine cancer, so see your primary care provider for an evaluation.

Neuroendocrine cancer treatment options

Treatment for neuroendocrine cancer depends on a variety of factors, including:

  • Where the tumor began
  • Whether the tumor is functional (functional NETs create and release hormones, which causes symptoms)
  • Tumor stage and grade
  • How fast the tumor is growing
  • Potential side effects
  • The patient’s goals and overall health
  • Somatostatin receptor status

Traditional treatment options for neuroendocrine cancer include:

  • Active surveillance: Closely monitoring the tumor and only starting treatment if it grows or spreads
  • Surgery: Removing the tumor and some nearby healthy tissue
  • Medications: Chemotherapy, somatostatin analogs, targeted therapy, immunotherapy, and peptide receptor radionuclide therapy (PRRT)
  • Radiation therapy: Using high-energy X-rays to destroy cancer cells

Researchers are studying new ways to treat neuroendocrine cancer or improve existing treatments, including:

  • Immunotherapy through new cancer vaccines and CAR T-Cell Therapy
  • Targeted treatments and combined treatments
  • Chemotherapy
  • PRRT
  • Palliative and supportive care to reduce symptoms and side effects

“For a disease as unusual as neuroendocrine cancer, working with a NET specialist is critical when deciding what treatments are required and if new techniques might improve both quality and quantity of life,” says Eric Liu, MD, Neuroendocrine Surgeon and Co-Director of the Neuroendocrine Program at Sarah Cannon Cancer Institute at Presbyterian/St. Luke’s Medical Center. “Certain treatments can only be used a limited number of times, so knowing which “tool to pull out of the toolbox” is vital.”

If you have questions about neuroendocrine cancer, call askSARAH at (844) 482-4812 or visit askSARAH online.