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.