Definition pancreatic NET (80-90 %) with excessively elevated secretion of VIP (vasoactive intestinal peptide)
original lit.:Verner et al. 1958 (Am J Med), Bloom et al. 1973 (Lancet) © www.gep-net.com / www.gep-net.de IncidenceIncidence: 0.05 - 0.2 - 0.5 per 1 Mill./year Pathology - Pathophysiology
mostly large and solitary tumors located in the pancreatic tail ( 50 % ) , frequently malignant ( 80 % )
extrapancreatic vipomas: carcinoids, lung cancer, pheochromo---cytoma, ganglioneuroma Diarrhea: VIP effect upon transport of elektrolytes and water - secondary hypokalemia Hypochlorhydria: inhibition of gastric acid secretion; flush: vasodilationClinical
Typical clinical syndrome
Verner-Morrison-Syn. - WDHA-Syn. - pancreatic "cholera"
1. watery diarrhea (WD) - 100 %; loss of 3 - 5 liters / day2. hypokalemia (H) - 100 %; < 3 mmol/l 3. achlorhydria (A) - 100 % other symptoms: weight loss, abdominal pain, colics, flush (urticaria) Diagnosis
Laboatory: elevated plasma conc. of VIP > 200 pg/ml, often 500 - 1000 pg/ml hypokalemia ( < 2.5 mmol/l), hypochlorhydria with normal pentagastrin stimulation, sometimes hypercalcemia, hyperglycemia frequent cosecretion of PP, calcitonin, GIP, secretin, prostaglandins Therapy-
Strategies
Surgical resection - surgery of liver (metastases) - chemoperfusion and -embolisation - chemotherapy symptoms : fluid supplementation (5 liters/day)- potassium substitution ( > 350 mval/day), somatostatin analogs, diarrhea: corticosteroids, clonidin, lithium, indomethacin, metoclopramid, loperamid Images