Zhang Jin-Ming, Zheng Chu-Wei, Li Xiao-Wen, Fang Zhi-Yun, Yu Mu-Xin, Shen Hai-Yan, Ji Xia
Department of Gastroenterology, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314001, Zhejiang Province, China.
Department of Pathology, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314001, Zhejiang Province, China.
World J Clin Cases. 2023 Sep 16;11(26):6223-6230. doi: 10.12998/wjcc.v11.i26.6223.
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.
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.
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.
卓-艾综合征（ZES）由胰腺或十二指肠神经内分泌肿瘤（通常称为胃泌素瘤）分泌过多胃泌素所致。高水平的胃泌素导致典型表现，包括水样腹泻和十二指肠多发溃疡。在此，我们报告了一例罕见的卓-艾综合征患者，其无高胃泌素血症且胃泌素瘤位置不典型。
一名72岁女性出现卓-艾综合征的典型临床表现，包括上腹痛、严重水样腹泻和酸性液体呕吐。然而，令人惊讶的是，她的血清胃泌素水平并未升高。此外，没有胃泌素瘤或任何其他致溃疡肿瘤的证据。进行了食管胃十二指肠镜检查以检查上消化道。考虑了修订诊断，并制定了个体化治疗方案。患者在经历间歇性、复发性卓-艾综合征发作时对抗酸药物有反应。18F-氟化铝-奥曲肽正电子发射断层扫描（18F-OC PET）/计算机断层扫描（CT）有助于定位肿瘤。术后病理和免疫组化结果表明该肿瘤是位于非传统部位的胃泌素瘤。
本病例研究证明了无高胃泌素血症患者出现类似卓-艾综合征表现的可能性。^{18}F-OC PET/CT是一种相对较新的成像技术，可用于诊断即使位置不典型的微小胃泌素瘤。
