Pancreatic neuroendocrine tumors: somatostatinoma
Are somatostatinomas hereditary?
Somatostatinomas occur less rarely in patients with neurofibromatosis (NF-1) than sporadically. NF-1 is one of the more common familial tumor syndromes. It is autosomal dominant, and occurs related to a genetic mutation of what is thought to be a tumor suppressor gene on chromosome 17. Tumors in NF-1 may involve both nervous and non-nervous system tissues. In adults, pheochromocytomas, GIST, breast cancer, and less commonly, somatostatinomas may occur in addition to the obvious neurofibromas. Somatostatinomas have been rarely seen in association with other disorders, such as von Hippel-Lindau, as well. Somatostatinomas in NF-1 are much more likely to be duodenal in origin.