Korean J Med.  2010 Sep;79(3):289-294.

A case of Zollinger-Ellison syndrome with MEN-1

• Kong YH
• Kim YD
• Han KH
• Lee SH
• Jung WJ
• Jang HJ
• Cheon GJ

Abstract

Zollinger-Ellison syndrome (ZES) is a clinical syndrome caused by excessive gastric acid secretion by gastrinoma, characteristically causing peptic disease and/or gastroesophageal reflux disease. Approximately one third of patients with gastrinoma have multiple endocrine neoplasia type 1 (MEN-1). A 56-year-old man was admitted for abdominal pain and diarrhea lasting for 2 weeks. The endoscopic findings revealed severe reflux esophagitis and multiple ulcers at the bulb and second portion of the duodenum. He was diagnosed as ZES based on typical clinical features such as markedly elevated fasting gastrin level (> or =1,263 pg/mL) and findings from a CT scan and somatostatin receptor scan. Pathologic findings after the operation revealed malignant gastrinoma. He was confirmed to have parathyroid adenoma and MEN-1. Despite antisecretory therapy with proton pump inhibitors, an esophageal stricture developed, and we performed esophageal balloon dilatation and stent insertion.

Keyword

Zollinger-Ellison syndrome; Gastrinoma; Multiple endocrine neoplasia (MEN-1)

MeSH Terms

Abdominal Pain
Diarrhea
Dilatation
Duodenum
Esophageal Stenosis
Esophagitis, Peptic
Fasting
Gastric Acid
Gastrinoma
Gastrins
Gastroesophageal Reflux
Humans
Middle Aged
Multiple Endocrine Neoplasia Type 1
Parathyroid Neoplasms
Proton Pump Inhibitors
Receptors, Somatostatin
Stents
Ulcer
Zollinger-Ellison Syndrome
Gastrins
Proton Pump Inhibitors
Receptors, Somatostatin