Neuroendocrine tumors of the gastro-entero-pancreatic system

Table 4 Clinical features of the main endocrine pancreatic tumors

Tumor (syndrome)	Clinical features and diagnostic tests	MEN-1 (%)	Metastases (%)	SnSRS (%)
Insulinoma	Spontaneous or fasting hypoglycemia (Whipple's triad)	8-10	10	50
	Positive fasting test (hypoglycemia with hyperinsulinism)
Gastrinoma (Zollinger-Ellison syndrome)	Peptic ulcers, diarrhea, GERD, BAO > 15 mEq/h	30	60	80
	Positive secretin test (serum gastrinemia > 200 ng/L within 10 min from secretin venous infusion, 2 U/kg per min)
VIP-oma (Verner Morrison syndrome)	Severe watery diarrhea (> 1L/die), hypokalemia, hypochlorhydria	Rare	70	80
Glucagonoma	Necrolytic migratory erythema, diabetes, weight loss, anemia, hypoaminoacidemia, venous thrombosis	Rare	60	80
Somatostatinoma	Diarrhea, steatorrhea, weight loss, diabetes, cholelithiasis	Not associated	84	80
CRH/ACTH-oma	Cushing's syndrome	-	90	-
GHRH-oma	Acromegaly	-	-	-

SnSRS: Sensitivity of ¹¹¹In-Pentetreotide scintigraphy (Octreoscan^®).