Duodenal neuroendocrine tumors: Classification, functional syndromes, diagnosis and medical treatment

Duodenal neuroendocrine tumors (NETs) comprise 2-3% of all GI endocrine tumors and are increasing in frequency. These include gastrinomas, somatostatinomas, nonfunctional NETs, gangliocytic paragangliomas, and poorly differentiated NE carcinomas. Although, the majority are nonfunctional, these tumors are a frequent cause of Zollinger-Ellison syndrome and can cause other clinical hormonal syndromes (carcinoid, Cushing's, etc.). In this chapter, their epidemiology, clinical aspects, localization, diagnosis and medical treatment are reviewed including the latest advances in each area.