An Unusual Presentation of Zollinger-Ellison Syndrome

Abstract

Zollinger-Ellison syndrome is an often progressive, persistent and frequently life-threatening disease, described for the first time as characterized by ulceration of the upper jejunum, hypersecretion of gastric acid and non-beta islet cell tumors of the pancreas; this syndrome is due to the hypersecretion of gastrin. We report a case of Zollinger-Ellison syndrome presenting as severe esophagitis evolving in stenosis, which demonstrates how a delayed diagnosis may induce risk of disease spreading. In this setting new diagnostic approaches, such as somatostatin receptor scanning and positron emission tomography with 68 Ga-labeled octreotide, could be particularly useful, as well as further new therapeutic options, such as molecular targeted treatments and peptide receptor radionuclide therapy, though surgery is currently the only form of curative treatment, and the role of the therapeutic options mentioned needs to be clarified by forthcoming studies.

Radiological Features of Zollinger–Ellison Syndrome: A Report of Two Cases

“…Gastrin causes excess production of gastric acid (hyperchlorhydria) which in turn results in severe recurrent ulcers of the esophagus, stomach, duodenum, and jejunum. 2 In total, 70% of gastrinomas are sporadic. Gastrinomas are associated with multiple endocrine neoplasia type 1 (MEN1) in 20 to 30% of cases.…”

“… 1 MEN1 is characterized by the occurrence of primary hyperparathyroidism (>90%), pancreaticoduodenal endocrine neoplasms (65–75%), and tumors of the anterior pituitary gland (30–65%). 2 ZES associated with MEN1 have a younger age at presentation, reduced incidence of peptic ulcer and GI bleeding, and a markedly increased incidence of renal calculi due to hyperparathyroidism. 3 In total, 65% of gastrinomas are malignant, and up to 30 to 40% of patients will have metastatic disease at initial presentation.…”

Zollinger-Ellison Syndrome: A Rare Case of Chronic Diarrhea

“…ZES is usually diagnosed in fifth decade of life. In one-third of patients with gastrinoma, metastatic disease is evident at the time of diagnosis [2]. Diarrhea and abdominal pain are the most common presenting symptoms of ZES [3].…”