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.
  • Tuberous sclerosis complex, which causes benign tumors in the brain, spinal cord, nerves, eyes, lungs, heart, kidneys and skin.
  • Von Hippel-Lindau syndrome, which can lead to tumors in many areas of the body, including the eyes, brain, uterus, kidneys, pancreas and adrenal glands. Although many of these tumors are benign (not cancerous) they can cause serious complications such as blindness, hearing loss and death. Patients with VHL also face increased risk for developing cancer in the kidneys and pancreas.
  • Neurofibromatosis, which causes nerve tissue tumors in several body areas. Lifelong monitoring, early cancer detection and timely treatment is essential for optimal survival and quality of life.
Roswell Park offers dedicated clinics to provide specialized care, monitoring and support for people with VHL or neurofibromatosis.

VHL care Neurofibromatosis care

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.

NET diagnosis