Vasoactive Intestinal Peptide Tumors

Overview

VIPomas (vasoactive intestinal peptide tumors) are rare cancers in which tumor cells arise from certain hormone-producing cells called islet cells. These islet cells are most often located in the pancreas, an organ that produces insulin and hormones that aid in digestion. However, these cells may also be located in or around the adrenal glands, which are hormone-secreting glands that are located just above each kidney that control the balance of sugar, salt, and water in the body.

In some cases, these VIPomas continue to produce excessive amounts of hormones, particularly one called vasoactive intestinal peptide. The excessive amount of this hormone causes the symptoms of the disease, of which watery diarrhea is the most prominent.

Because they develop in islet cells, VIPomas are also referred to as a type of islet cell tumor. When symptoms are present, VIPoma is also known as Verner-Morrison syndrome, a name based on the 2 doctors who were the first to describe the condition.

In later stages of the disease, VIPomas may spread (metastasize) to other organs.

Who Is More At Risk

VIPomas are very rare cancers. Each year very few new cases of VIPoma are reported (0.05 to 0.2 cases per million adults). Even fewer cases in children are reported.

When VIPomas occur in adults, they appear most commonly between the ages of 40 and 50 years and they usually develop in the pancreas. In children, VIPomas more commonly arise in or near the adrenal gland.

A person may have an increased risk of developing a VIPoma if they have a family history of multiple endocrine neoplasia syndrome type 1 (MEN 1), a hereditary condition that increases the risk of developing tumors of certain hormone-producing glands.