Anlauf Martin, Garbrecht Nele, Henopp Tobias, Schmitt Anja, Schlenger Regina, Raffel Andreas, Krausch Markus, Gimm Oliver, Eisenberger Claus F, Knoefel Wolfram T, Dralle Henning, Komminoth Paul, Heitz Philipp U, Perren Aurel, Klöppel Gunter
Department of Pathology, University of Kiel, Michaelisstrasse 11, 24105 Kiel, Germany.
World J Gastroenterol. 2006 Sep 14;12(34):5440-6. doi: 10.3748/wjg.v12.i34.5440.
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 Zurich, 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 expressing gastrin, but not as gastrinomas.
胃泌素瘤被定义为与卓-艾综合征（ZES）相关的分泌胃泌素的肿瘤。ZES的特征是空腹血清胃泌素水平升高、促胰液素刺激试验阳性以及诸如复发性消化性溃疡病、胃食管反流病和偶尔腹泻等临床症状。在遗传学上，非遗传性（散发性）胃泌素瘤与遗传性胃泌素瘤不同，后者与1型多发性内分泌腺瘤病（MEN1）综合征相关。一般来说，散发性十二指肠胃泌素瘤体积小且为单发，而遗传性十二指肠胃泌素瘤则多发。散发性胃泌素瘤发生于十二指肠或胰腺，而遗传性胃泌素瘤几乎都发生于十二指肠。我们的77例散发性十二指肠神经内分泌肿瘤（NETs）系列中，有18例患者（23.4%）患有胃泌素瘤和ZES。在从瑞士苏黎世和德国基尔的病理科NET档案中收集的535例散发性胰腺NETs中，24例患者（4.5%）患有散发性胰腺胃泌素瘤和ZES。这些NETs必须与胃泌素免疫组化阳性但无ZES证据的肿瘤相区分。另有19例患者患有MEN1和ZES。这些患者仅表现为十二指肠胃泌素瘤，而非胰腺胃泌素瘤。散发性和MEN1相关的十二指肠胃泌素瘤的预后优于胰腺胃泌素瘤，因为它们进展为肝转移的速度较慢。总之，散发性和MEN1相关的十二指肠和胰腺胃泌素瘤表现出不同的临床病理和遗传特征。散发性十二指肠产生胃泌素肿瘤的发病率正在上升，可能是由于诊断程序的优化。相比之下，胰腺MEN1相关的胃泌素瘤似乎极为罕见。相当一部分胃泌素免疫组化表达但无ZES证据的肿瘤应被指定为表达胃泌素的无功能NETs，而不是胃泌素瘤。
