At a Glance Vipoma (Verner-Morrison syndrome, pancreatic cholera, WDHA syndrome – for watery diarrhea, hypokalemia, and achlorhydria) is one of several rare types of functioning pancreatic neuroendocrine tumors (NETs) and accounts for 2-4% of all pancreatic NETs. Most cases are sporadic, but about 10% are associated with multiple endocrine neoplasia type 1 (MEN1). Vipomas are…
At a Glance Carcinoids are rare, slowly growing subtypes of neuroendocrine tumors (NETs). They arise most commonly within the gastrointestinal (65% of cases) or respiratory (35% of cases) systems and are characterized by serotonin (5-hydroxytryptamine) production. The carcinoid syndrome occurs in about 10% of carcinoid tumors (usually in the presence of metastatic disease to the…
At a Glance Clinical manifestations of Zollinger-Ellison syndrome (ZES), a syndrome caused by a gastrin-secreting neuroendocrine tumor (gastrinoma), result from high gastric acid output due to hypertrophy of parietal and histamine-secreting enterochromaffin-like (ECL) cells, as well as direct stimulation of parietal cells. More than 90% of patients develop peptic ulcers, but only a small number…
At a Glance Glucagonoma (“sweet” syndrome) is one of the functioning pancreatic neuroendocrine tumors (NETs) that account for 1-3% of all pancreatic NETs. Most cases are sporadic, but 5-17% are associated with multiple endocrine neoplasia type 1 (MEN1). Glucagonomas are typically single, large tumors (average size 6 cm) and are found almost exclusively in the…
At a Glance Insulinomas are the most common functioning pancreatic neuroendocrine tumors (NETs) and account for 20-30% of all pancreatic NETs. Most cases are sporadic, but about 5-10% are associated with multiple endocrine neoplasia type 1 (MEN1). The tumors are usually solitary (except in patients with MEN1), small (<1 cm), and almost exclusively intrapancreatic. In…
