Diseases of the Endocrine Pancreas

Neuroendocrine Tumors (NET) of the Pancreas/Duodenum
These are rare tumors. They are most often sporadic (not inherited), but some may be part of inherited syndromes such as MEN or Von Hippel-Lindau.

Nonfunctioning NETs of the Pancreas
These tumors do not produce hormones that cause symptoms. Therefore they are usually discovered late. If the disease has not spread outside of the pancreas, the best treatment is usually surgery.

Gastrinoma
These tumors produce gastrin, which can lead to ulcers and diarrhea, known as Zollinger-Ellison syndrome. The tumors are found in the “gastrinoma triangle”, which encompasses the duodenum and the head of the pancreas. They sometimes spread to lymph nodes, liver and other organs. Gastrinomas may be part of the MEN1 syndrome.

Insulinoma
These tumors produce insulin, which leads to profound hypoglycemia and symptoms of mental status changes, and loss of consciousness. The symptoms disappear with eating sugary foods. Most (90%) are benign. They are often difficult to diagnose and localize. Insulinomas may be part of the MEN1 syndrome.

Other hormone producing pancreatic NETs
Additional very rare tumors include somatostatinomas, VIPomas, and glucagonomas.