Functional Pancreatic Neuroendocrine Tumors

Overview

Definition: Gastrin-secreting tumor of the endocrine pancreas; can also occur in the duodenum
Function: Induces gastric acid secretion
Symptoms and Signs: Recurrent ulcers in the stomach, duodenum, and sometimes jejunum; abdominal pain, diarrhea
Diagnosis: Ulcers not responding to conventional therapy; high blood gastrin levels; not suppressed by secretin
Treatment: Somatostatin (octreotide), surgical resection
Associated Syndrome: Zollinger-Ellison syndrome

Definition: Insulin-secreting tumor from the beta cells of the endocrine pancreas
Function: Causes hypoglycemia due to excess insulin
Symptoms and Signs: Hunger, nervousness, sweating, lethargy, confusion, coma
Diagnosis: Whipple's triad (hypoglycemia, symptoms of hypoglycemia, symptom relief with glucose); blood work (low glucose, high insulin, high C peptide)
Treatment: Surgical resection

Definition: Glucagon-secreting tumor from the alpha cells of the endocrine pancreas
Function: Causes increased glucose
Symptoms and Signs: Mild diabetes, dermatitis (necrolytic migratory erythema), weight loss, anemia, venous thrombosis (DVT)
Diagnosis: Increased glucagon levels
Treatment: Somatostatin (octreotide), surgical resection

Also Known As: Verner-Morrison syndrome
Definition: VIP-secreting tumor from the D1 cells of the endocrine pancreas
Function: Excessive secretion of water and electrolytes (VIP)
Symptoms and Signs: Explosive watery diarrhea, low hydrochloric acid, low potassium, dehydration
Treatment: Somatostatin (octreotide), surgical resection

Definition: Somatostatin-secreting tumor from the D cells of the endocrine pancreas
Function: Inhibits multiple hormones
Symptoms and Signs: Mild diabetes, gall stones, steatorrhea, hypochlorhydria (low hydrochloric acid)
Treatment: Somatostatin (octreotide), surgical resection