Gastrinoma and Zollinger Ellison syndrome:A roadmap for the management between new and old therapies  

作　　者：Roberta Elisa Rossi Alessandra Elvevi Davide Citterio Jorgelina Coppa Pietro Invernizzi Vincenzo Mazzaferro Sara Massironi 

机构地区：[1]HPB Surgery,Hepatology and Liver Transplantation,ENETS Center of Excellence,Fondazione IRCCS Istituto Nazionale Tumori(INT,National Cancer Institute),Milan 20133,Italy [2]Department of Pathophysiology and Transplantation,University of Milan,Milan 20122,Italy [3]Division of Gastroenterology and Center for Autoimmune Liver Diseases,Department of Medicine and Surgery,University of Milano-Bicocca,Monza 20900,Italy [4]European Reference Network on Hepatological Diseases(ERN RARE-LIVER),San Gerardo Hospital,Monza 20033,Italy [5]Department of Oncology and Hemato-Oncology,University of Milan,Milan 20122,Italy

出　　处：《World Journal of Gastroenterology》2021年第35期5890-5907,共18页世界胃肠病学杂志（英文版）

摘　　要：Zollinger-Ellison syndrome(ZES)associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion,which typically leads to gastroesophageal reflux disease,recurrent peptic ulcers,and chronic diarrhea.As symptoms of ZES are nonspecific and overlap with other gastrointestinal disorders,the diagnosis is often delayed with an average time between the onset of symptoms and final diagnosis longer than 5 years.The critical step for the diagnosis of ZES is represented by the initial clinical suspicion.Hypergastrinemia is the hallmark of ZES;however,hypergastrinemia might recognize several causes,which should be ruled out in order to make a final diagnosis.Gastrin levels>1000 pg/mL and a gastric pH below 2 are considered to be diagnostic for gastrinoma;some specific tests,including esophageal pH-recording and secretin test,might be useful in selected cases,although they are not widely available.Endoscopic ultrasound is very useful for the diagnosis and the local staging of the primary tumor in patients with ZES,particularly in the setting of multiple endocrine neoplasia type 1.Some controversies about the management of these tumors also exist.For the localized stage,the combination of proton pump inhibitory therapy,which usually resolves symptoms,and surgery,whenever feasible,with curative intent represents the hallmark of gastrinoma treatment.The high expression of somatostatin receptors in gastrinomas makes them highly responsive to somatostatin analogs,supporting their use as anti-proliferative agents in patients not amenable to surgical cure.Other medical options for advanced disease are super-imposable to other neuroendocrine neoplasms,and studies specifically focused on gastrinomas only are scant and often limited to case reports or small retrospective series.The multidisciplinary approach remains the cornerstone for the proper management of this composite disease.Herein,we reviewed available literature about gastrinoma-associated ZES with a specific focus on differential diagnosis,pro

关 键 词：GASTRINOMA Zollinger-Ellison syndrome Neuroendocrine neoplasms Pancreatic neuroendocrine neoplasm Duodenal neuroendocrine neoplasm Diagnosis Therapy 

分 类 号：R735.2[医药卫生—肿瘤]