What Causes Neuroendocrine Cancer?

Most neuroendocrine tumors appear to occur at random, without any known cause or risk factors. However, some people with neuroendocrine tumors have inherited genetic mutations that increase their risk for certain cancers. Some inherited genetic syndromes are considered risk factors for neuroendocrine tumors. These syndromes include:

  • Multiple Endocrine Neoplasia (MEN) type 1 which is associated with tumors in the parathyroid, pituitary, lungs, pancreas and other gastrointestinal sites
  • Multiple Endocrine Neoplasia (MEN) type 2 which is associated with medullary thyroid cancer, pheochromocytoma and excess parathyroid hormone.
  • Von Hippel-Lindau syndrome
  • Tuberous sclerosis complex
  • Neurofibromatosis

In addition, certain conditions that affect the body’s ability to produce stomach acid may increase risk for NETs of the stomach. These conditions include:

  • Atrophic gastritis
  • Pernicious anemia
  • Zollinger-Ellison syndrome

If you have one of these risk factors, your doctor may watch you closely. If you are at extremely high risk, your doctor may recommend removing an organ, such as the thyroid, to prevent a neuroendocrine tumor from growing there.