Neuroendocrine GEP-Tumors (GEP-NET)


(Zollinger-Ellison-Syndrome / ZES)

pancreatic NET (50-85 %) with excessively elevated secretion of gastrin, extrapancreatic localisation, e.g. duodenum
original lit.:
Zollinger et al. 1955 (Ann Surg), Ellison et al. 1956 (Surgery)
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Incidence 0.5 - 1.5 per 1 Mill./year

Pathology - Pathophysiology

small pancreatic and extrapancreatic tumors ( < 10 mm); frequently multiple tumors; frequently malignant
Ulcers: elevated secretion of gastric acid: pain, bleeding, perforation
Diarrhea: denaturation of proteins (gastric acid), inactivation of pancreatic lipase: steatorrhea, maldigestion (bile acids)
chronic hypergastrinemia in gastrinoma and due to chronic-atrophic gastritis (pernicious anemia) with hypo- or achlorhydria (reduced gastric acid) may be the cause of carcinoid developement in the stomach.

Typical clinical syndrome

Zollinger - Ellison - Syndrome (ZES):

1. peptic ulcers of stomach, duodenum, with typ. complications
2. diarrhea, watery (secretory) or steatorrhea
other symptoms: maldigestion, carcinoids of stomach


Laboratory: Hypergastrinemia > 300 pg/ml, often > 1000 pg/ml (G-34 "big gastrin");
elevated gastric acid output (BAO - basic acid output) > 15 to > 30 mval/h., pH < 2.5; Provocation: Secretin iv.
Differential diagnosis hypergastrinemia: antral G-cell-hyperplasia, chronic atrophic gastritis (pernicious anemia: 500-1000 pg/ml), vagotomy, Billroth-II-surgery, renal insufficiency
surgical resection - surgery of lymph nodes and liver (metastases) - chemoperfusion and -embolisation - chemotheray
Symptoms: blockers of proton pump, high-dose with control of gastrin levels (titration); somatostatin-analogs rarely effective, gastrectomy obsolete