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Etiology of neuroendocrine tumors

Besides a few, very rare hereditary cases, the cause is unknown. As for most other tumor types, it is assumed that a combination of genetic dispositions and exogenous factors play a role.

In multiple endocrine neoplasia (MEN), von Hippel-Lindau disease and Neurfibromatosis type l, one finds mutations in sigle genes that causes a variety of neuroendocrine tumors in various organs of the same patient (3).

  • In MEN (mutation in the MEN gene), the organs which are most often involved are the pancreas, parathyroid gland, pituitary gland, and adrenal glands.
  • In von Hippel-Lindau disease (mutation in VHL gene), the pancreas and adrenal glands are involved, among others
  • For neurofibromatosis type I, neuroendocrine tumors are found in the pancreas and duodenum. 

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