Pancreatic Neuroendocrine Tumors – insert in Pathoma endocrine

 Insulinoma
 Most common type of pancreatic neuroendocrine tumor – episodic hypoglycemia precipitated by fasting
or exercise and relieved by feeding or parenteral administration of glucose
 90% are benign
 Morphology:
o Look just like giant islets – preserved architecture
o Amyloid deposits are characteristic
 Hyperinslunism can also be caused by focal or diffuse hyperplasia of islets – can be seen in infants whose
mothers had diabetes during pregnancy, in Beckwith-Wiedemann syndrome, and in a rare mutation in B-
cell K-channel protein
 Hypoglycemia is usually mild and many patients have no symptoms
 Surgical removal resolves the syndrome
 Note: retroperitoneal fibromas and fibrosarcomas can produce insulin ectopically and thus cause
hypoglycemia
 Gastrinoma
 Can arise in pancreas but also in duodenum and peripancreatic fat
 more than half are locally invasive or have metastasized at diagnosis
 in 25% of patients, arise in association with MEN1
 Zollinger-Ellison Syndrome:
o Hypergastrinemia causes peptic ulcers due to high gastric acid secretion – these are unresponsive
to therapy
o Can arise in unusual locations like the jejunum
 More than 50% of patients have diarrhea – 30% patients present with diarrhea
 Treat by suppressing gastric acid secretion and resection of the tumor
 Liver metastases seriously reduce life expectancy
 Glucagonoma
 Alpha cell tumor
 Syndrome of mild DM, necrolytic migratory erythema [image below (a blistering red rash)], and anemia

 Frequent in perimenopausal and menopausal women

 Somatostatinoma
 DM, hypochloridria, cholelithiasis, and steatorrhea
 VIPoma
 Watery diarrhea, hypokalemia, achlorydia
 Some of these are locally invasive and metastatic
 Neural crest tumors like pheochromocytoma are associated with these
 Pancreatic polypeptide secreting tumors present with mass effect without any endocrine Sx