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首页> 外文期刊>Medical science monitor : >Case presentation of gastrinoma combined with gastric carcinoid with the longest survival record - Zollinger-Ellison syndrome: pathophysiology, diagnosis and therapy
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Case presentation of gastrinoma combined with gastric carcinoid with the longest survival record - Zollinger-Ellison syndrome: pathophysiology, diagnosis and therapy

机译：胃泌素瘤合并胃类癌具有最长生存记录的病例报告-Zollinger-Ellison综合征：病理生理，诊断和治疗

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BACKGROUND: Zollinger-Ellison syndrome is a very rare disease caused bytumor with gastrin producing cells accompanied by hypergastrinemia leading to gastric hypersecretionand peptic ulcers and their complications.CASE STUDY: Female case of gastrinoma (Zollinger-Ellison syndrome;Z-E) with a record of 38 yrs of survival. Acute gastro-duodenal ulcers started at 28 yr of age and Z-Ewas diagnosed by using gastrin assays. Basal and maximal acid outputs and ratio of basal/maximal outputswere away over normal limits. Because of ulcer recurrence and complications, patient was subjected toseveral gastric surgeries but refused total gastrectomy. She was also treated with many H2-receptor (R)antagonists and proton-pump inhibitors (PPI), each new drug being initially highly effective but thenshowing declining efficacy except when PPI, lansoprazole was used. The gastrin level rose in the courseof disease from initial high value of 2000 pg/mL to the extreme 4500 ng/mL at present. During the last2 yrs, metastasis mainly to liver developed and they were successfully treated by synthetic octapeptidederivative of somatostatin and, as a result, metastatis partly reduced and plasma gastrin drasticly decreased.Biopsy taken from liver metastasis showed the presence of typical gastrinoma cells with gastrin and chromogranin,while that from oxyntic mucosa revealed the ECL-cell hyperplasia with carcinoid tumors and unexpectedgastric atrophy.CONCLUSIONS: This phenomenal case described in this article might be the new proven evidenceneeded by gastroenterologists to overturn the traditional treatment using total gastrectomy as a treatmentof choice to the partial gastrectomy combined with proton pump inhibitors.
机译：背景：Zollinger-Ellison综合征是一种由胃泌素产生细胞引起的肿瘤伴有高胃泌素血症导致胃分泌过多和消化性溃疡及其并发症的罕见疾病。案例研究：女性胃泌素瘤病例（Zollinger-Ellison综合征; ZE）有38的记录生存之年。急性胃十二指肠溃疡始于28岁，并通过使用胃泌素测定法诊断为Z-E。基本和最大酸产量以及基本/最大产量之比超出正常范围。由于溃疡复发和并发症，患者接受了几次胃外科手术，但拒绝进行全胃切除术。她还接受了许多H2受体拮抗剂和质子泵抑制剂（PPI）的治疗，每种新药最初都非常有效，但除使用PPI兰索拉唑外，其他药物的疗效均下降。在疾病过程中，胃泌素水平从最初的2000 pg / mL的最高值上升到目前的最高4500 ng / mL。在过去的2年中，转移主要发生在肝脏，并通​​过生长抑素的合成八肽衍生物成功治疗，结果转移灶部分减少，血浆胃泌素急剧下降。肝转移活检显示典型的胃泌素瘤细胞存在胃泌素和嗜铬粒蛋白，而来自氧化性黏膜的嗜铬粒蛋白则显示出ECL细胞增生，并伴有类癌肿瘤和意外的胃萎缩。结论：本文描述的这一惊人案例可能是胃肠病学家们需要证明的新证据，以推翻传统治疗方法，即采用全胃切除术作为治疗的选择部分胃切除术结合质子泵抑制剂。

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