背景:Zollinger-Ellison综合征(ZES)是胰腺或十二指肠神经内分泌肿瘤胃泌素分泌过多所致,通常被称为胃泌素瘤。高水平的胃泌素导致典型的表现,包括十二指肠中的水样腹泻和多发性溃疡。这里,我们介绍了一例罕见的ZES患者,没有高胃泌素血症以及胃泌素瘤的不典型部位.
方法:一名72岁女性,表现为典型的ZES临床表现,包括上腹痛,严重的水样腹泻,和酸性液体呕吐物。令人惊讶的是,然而,她的血清胃泌素水平没有升高。此外,没有胃泌素瘤或任何其他溃疡性肿瘤的证据。进行食管胃十二指肠镜检查以检查上消化道。考虑了修改后的诊断,并制定了个体化治疗方案。患者在经历间歇性时对抗酸药物有反应,反复发作的ZES。18F-AlF-NOTA-奥曲肽正电子发射断层扫描(18F-OCPET)/计算机断层扫描(CT)有助于定位肿瘤。术后病理和免疫组织化学结果表明肿瘤是位于非常规部位的胃泌素瘤。
结论:本病例研究证明了在没有高胃泌素血症的患者中ZES样表现的可能性。18F-OCPET/CT是一种相对较新的成像技术,可用于诊断甚至在位置上非典型的微小胃泌素瘤。
BACKGROUND: Zollinger-Ellison syndrome (ZES) results from hypersecretion of gastrin from pancreatic or duodenal neuroendocrine tumors, commonly referred to as gastrinomas. The high levels of gastrin lead to a typical presentation involving watery diarrhea and multiple ulcers in the duodenum. Here, we have presented the rare
case of a patient with ZES and absence of hypergastrinemia as well as an atypical location of gastrinoma.
METHODS: A 72-year-old woman presented with the typical clinical manifestations of ZES, including upper abdominal pain, significant watery diarrhea, and acidic liquid vomitus. Surprisingly, however, she did not have an increased level of serum gastrin. In addition, there was no evidence of gastrinoma or any other ulcerogenic tumor. Esophagogastroduodenoscopy was conducted to examine the upper digestive tract. Revised diagnoses were considered, and an individualized treatment plan was developed. The patient responded to antacid medication while experiencing intermittent, recurring bouts of ZES. 18F-AlF-NOTA-octreotide positron emission tomography (18F-OC PET)/computed tomography (CT) helped locate the tumor. Postoperative pathology and immunohistochemistry results suggested that the tumor was a gastrinoma located at an unconventional site.
CONCLUSIONS: This present
case study demonstrates the possibility of ZES-like manifestation in patients with absence of hypergastrinemia. 18F-OC PET/CT is a relatively new imaging technique that can be applied for diagnosing even tiny gastrinomas that are atypical in terms of location.