Sporadic versus hereditary gastrinomas of the duodenum and pancreas: Distinct clinico-pathological and epidemiological features  

作　　者：Martin Anlauf Nele Garbrecht Tobias Henopp Anja Schmitt Regina Schlenger Andreas Raffel Markus Krausch Oliver Gimm Claus F Eisenberger Wolfram T Knoefel Henning Dralle Paul Komminoth Philipp U Heitz Aurel Perren Günter Klppel 

机构地区：[1]Department of Pathology, Universityof Kiel, Germany [2]Department of Pathology, University of Zürich, Switzerland [3]Department of Pathology, Universityof Kiel, Germany Department of Forensic Medicine, Universityof Kiel, Germany [4]Department of General, Visceral andPediatric Surgery, University of Düsseldorf, Germany [5]Department of General andVisceral Surgery, University of Halle-Wittenberg, Germany

出　　处：《World Journal of Gastroenterology》2006年第34期5440-5446,共7页世界胃肠病学杂志（英文版）

基　　金：Supported by the Hensel Stiftung Kiel (F370011, MA and GK),the Swiss National Foundation (SNF 31-61884, AP and PK) andthe German Society of Pathology (MA). Tobias Henopp has afellowship sponsored by Ipsen GMBH, Ettlingen, Germany

摘　　要：Gastrinomas are defined as gastrin secreting tumors that are associated with Zollinger-Ellison syndrome （ZES）. ZES is characterized by elevated fasting gastrin serum levels, positive secretin stimulation test and clinical symptoms such as recurrent peptic ulcer disease, gastroesophageal reflux disease and occasional diarrhea. Genetically, nonhereditary （sporadic） gastrinomas are distinguished from hereditary gastrinomas, which are associated with multiple endocrine neoplasia type 1 （MEN1） syndrome. In general, duodenal gastrinomas are small and solitary if they are sporadic and multiple as well as hereditary. The sporadic gastrinomas occur in the duodenum or in the pancreas while the hereditary gastrinomas almost all occur in the duodenum. Our series of 77 sporadic duodenal neuroendocrine tumors （NETs） includes 18 patients （23.4%） with gastrinomas and ZES. Of 535 sporadic NETs in the pancreas collected from the NET archives of the departments of pathology in Zürich, Switzerland, and Kiel, Germany, 24 patients （4.5%） suffered from sporadic pancreatic gastrinomas and ZES. These NETs have to be distinguished from tumors with immunohistochemical positivity for gastrin but without evidence of ZES. An additional 19 patients suffered from MEN1 and ZES. These patients showed exclusively duodenal gastrinomas, but not pancreatic gastrinomas. The prognosis of sporadic and MEN1- associated duodenal gastrinomas is better than that of pancreatic gastrinomas, since they progress slowly to liver metastasis. In summary, sporadic and MEN1- associated gastrinomas in the duodenum and pancreas show different clinico-pathological and genetic features. The incidence of sporadic duodenal gastrin-producing tumors is increasing, possibly due to optimized diagnostic procedures. In contrast, pancreatic MEN1- associated gastrinomas seem to be extremely rare. A considerable subset of tumors with immunohistochemical expression of gastrin but without evidence of ZES should be designated as functionally inactive NETs expGastrinomas are defined as gastrin secreting tumors that are associated with Zollinger-Ellison syndrome (ZES). ZES is characterized by elevated fasting gastrin serum levels, positive secretin stimulation test and clinical symptoms such as recurrent peptic ulcer disease, gastroesophageal re?ux disease and occasional diarrhea. Genetically, nonhereditary (sporadic) gastrinomas are distinguished from hereditary gastrinomas, which are associated with multiple endocrine neoplasia type 1 (MEN1) syndrome. In general, duodenal gastrinomas are small and solitary if they are sporadic and multiple as well as hereditary. The sporadic gastrinomas occur in the duodenum or in the pancreas while the hereditary gastrinomas almost all occur in the duodenum. Our series of 77 sporadic duodenal neuroendocrine tumors (NETs) includes 18 patients (23.4%) with gastrinomas and ZES. Of 535 sporadic NETs in the pancreas collected from the NET archives of the departments of pathology in Zürich, Switzerland, and Kiel, Germany, 24 patients (4.5%) suffered from sporadic pancreatic gastrinomas and ZES. These NETs have to be distinguished fromtumors with immunohistochemical positivity for gastrin but without evidence of ZES. An additional 19 patients suffered from MEN1 and ZES. These patients showed exclusively duodenal gastrinomas, but not pancreatic gastrinomas. The prognosis of sporadic and MEN1- associated duodenal gastrinomas is better than that of pancreatic gastrinomas, since they progress slowly to liver metastasis. In summary, sporadic and MEN1- associated gastrinomas in the duodenum and pancreas show different clinico-pathological and genetic features. The incidence of sporadic duodenal gastrin-producing tumors is increasing, possibly due to optimized diagnostic procedures. In contrast, pancreatic MEN1- associated gastrinomas seem to be extremely rare. A considerable subset of tumors with immunohistochemical expression of gastrin but without evidence of ZES should be designated as functionally inactive NETs expressing gastrin, but not a

关 键 词：Endocrine tumor GASTRINOMA Multiple endocrine neoplasia type 1 Precursor lesion Zollinger-Ellison syndrome 

分 类 号：R735[医药卫生—肿瘤]