The exact cause of neuroendocrine tumors isn't known. These cancers begin in neuroendocrine cells that have traits similar to those of nerve cells and hormone-producing cells. Neuroendocrine cells are found throughout your body.
Neuroendocrine tumors begin when neuroendocrine cells develop changes (mutations) in their DNA. The DNA inside a cell contains the instructions that tell the cell what to do. The changes tell the neuroendocrine cells to multiply rapidly and form a tumor.
Some neuroendocrine tumors grow very slowly. Others are aggressive cancers that invade and destroy normal body tissue or spread (metastasize) to other parts of the body.
The risk of neuroendocrine tumors is higher in people who inherit genetic syndromes that increase the risk of cancer. Examples include:
• Multiple endocrine neoplasia, type 1 (MEN 1)
• Multiple endocrine neoplasia, type 2 (MEN 2)
• Von Hippel-Lindau disease
• Tuberous sclerosis
Courtesy: Mayo Clinic