Causes

It's not clear what causes most pancreatic neuroendocrine tumors.

Pancreatic neuroendocrine tumors occur when hormone-producing cells in the pancreas (islet cells) develop changes (mutations) in their DNA — the material that provides instructions for every chemical process in your body. DNA mutations cause changes in these instructions. One result is that cells may begin to grow out of control and eventually form a tumor — a mass of cancerous cells. Sometimes the cancer cells break away and spread to other organs, such as the liver.

Some inherited syndromes increase the risk of this type of cancer, including:

• Multiple endocrine neoplasia, type 1 (MEN 1) syndrome
• Von Hippel-Lindau disease
• Von Recklinghausen's disease (neurofibromatosis 1)
• Tuberous sclerosis