VIPomas, glucagonomas, and somatostatinomas are three rare kinds of neuroendocrine tumors with an estimated incidence of less than 1 per 10 million patient years. VIPomas are mainly localized in the pancreas and VIP secretion can cause severe diarrhea. Glucagonomas cause hyperglycemia, necrolytic migratory erythema, cachexia, and glottitis. The somatostatinoma syndrome is characterized by diabetes mellitus of recent onset, decreased gastric acid secretion, cholelithiasis, steatorrhea, and anemia/weight loss. Treatment of these neuroendocrine tumors is surgical if a radical resection is feasible. Metastatic tumor is treated symptomatically and often somatostatin analogues are very effective.

Functional neuroendocrine tumor, Glucagonoma, Neuroendocrine tumor, Secretory syndromes, Somatostatinoma, VIPoma,
Erasmus MC: University Medical Center Rotterdam

Zandee, W.T, & de Herder, W.W. (2018). VIPoma, glucagonoma, and somatostatinoma. In Encyclopedia of Endocrine Diseases (pp. 52–57). doi:10.1016/B978-0-12-801238-3.64340-4