|Name||Zollinger-Ellison syndrome (ZES)|
|Description||Zollinger-Ellison syndrome (ZES) is a rare endocrinopathy caused by tumors of the pancreas and duodenum. These tumors, called gastrinomas, release gastrin to produce large amounts of acid that result in severe gastroesophageal peptic ulcer disease and diarrhea. Most ZES cases are sporadic, but about over 20 percent are caused by an inherited genetic disorder called multiple endocrine neoplasia type 1 (see H00247). The clinical presentation is not specific for this disease and there is overlap of symptoms similar to those of a peptic ulcer. The most common symptoms include abdominal pain and diarrhea, sometimes accompanied by heartburn, nausea, and weight loss. Peptic ulceration complicated by bleeding is present in 25% of patients, and is more frequently in patients with sporadic ZES than in those with MEN1. In addition, the gastrinomas may be cancerous. The cancer can be spread to other parts of the body, most commonly to regional lymph nodes and the liver. The treatment of the ZES includes surgical removal and medical management of gastric acid hypersecretion for the prevention of malignant transformation and the genesis of complications.|
|Category||Endocrine disease; Cancer|
|Gene||MEN1 [HSA:4221] [KO:K14970]|
|Marker||Serum gastrin [HSA:2520]|
|Drug||Omeprazole [DG:DG00020] Rabeprazole [DG:DG00022] Pantoprazole [DG:DG00021] Lansoprazole [DR:D00355] Esomeprazole [DG:DG00023] (Proton pump inhibitors)
Cimetidine [DG:DG00295] Ranitidine [DG:DG00018] Famotidine [DR:D00318] (H2-receptor antagonist)
|Other DBs||ICD-10: E16.4
Ito T, Igarashi H, Uehara H, Jensen RT
Pharmacotherapy of Zollinger-Ellison syndrome.
Expert Opin Pharmacother 14:307-21 (2013)
Gibril F, Schumann M, Pace A, Jensen RT
Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature.
Medicine (Baltimore) 83:43-83 (2004)
PMID:24319020 (description and diagnosis)
Epelboym I, Mazeh H
Zollinger-Ellison syndrome: classical considerations and current controversies.
Oncologist 19:44-50 (2014)
Wilcox CM, Hirschowitz BI
Treatment strategies for Zollinger-Ellison syndrome.
Expert Opin Pharmacother 10:1145-57 (2009)
Nieto JM, Pisegna JR
The role of proton pump inhibitors in the treatment of Zollinger-Ellison syndrome.
Expert Opin Pharmacother 7:169-75 (2006)