Pellicano R, De Angelis C, Resegotti A, Rizzetto M
Department of Gastro-Hepatology, San Giovanni Battista (Molinette) Hospital, Turin, Italy.
Panminerva Med. 2006 Mar;48(1):33-40.
Zollinger-Ellison syndrome (ZES) is characterised by peptic ulcers of the upper gastrointestinal tract failing to heal despite maximal medical therapy, diarrhoea and marked gastric acid hypersecretion associated with a gastrin-secreting tumour (gastrinoma). ZES might be associated with multiple endocrine neoplasia type 1. The main diagnostic features are hypergastrinemia and acid hypersecretion. When these parameters give borderline results, provocation test (with secretin or calcium) may be required. To identify the localisation of gastrinoma several imaging techniques have been proposed. Somatostatin receptor scintigraphy is capable to localise the tumour in 80% of the cases and to identify it even in anatomic sites other than pancreas and duodenum. Endoscopic ultrasonography has a sensitivity as high as 79-93% and a specificity of 93%. The 2 main principal therapeutic strategies are to control both the gastric acid hypersecretion and the growth of the neoplasia. Proton pump inhibitors (PPIs) are the drugs of choice for patients with ZES. Furthermore, safety of PPIs in the maintenance therapy has been proven both in short- and in long-term studies. The best surgical treatment is excision of gastrinoma before metastatic spread has occurred. Somatostatin-analogues can reduce both gastric acid hypersecretion and serum gastrin levels. Moreover, they have an antiproliferative effect. Chemotherapy, interferon and embolisation are indicated in rapidly evolving tumours or in cases in which the tumoral symptoms cannot be treated by other approaches.
卓-艾综合征（ZES）的特征是尽管接受了最大程度的药物治疗，上消化道消化性溃疡仍无法愈合、腹泻以及与胃泌素分泌肿瘤（胃泌素瘤）相关的明显胃酸分泌过多。ZES可能与1型多发性内分泌肿瘤有关。主要诊断特征是高胃泌素血症和胃酸分泌过多。当这些参数给出临界结果时，可能需要进行激发试验（使用促胰液素或钙剂）。为了确定胃泌素瘤的定位，已经提出了几种成像技术。生长抑素受体闪烁扫描能够在80%的病例中定位肿瘤，甚至能在胰腺和十二指肠以外的解剖部位发现肿瘤。内镜超声检查的敏感性高达79% - 93%，特异性为93%。两种主要的治疗策略是控制胃酸分泌过多和肿瘤的生长。质子泵抑制剂（PPIs）是ZES患者的首选药物。此外，短期和长期研究均已证实PPIs在维持治疗中的安全性。最佳的手术治疗是在转移扩散发生之前切除胃泌素瘤。生长抑素类似物可以降低胃酸分泌过多和血清胃泌素水平。此外，它们还有抗增殖作用。化疗、干扰素和栓塞适用于快速进展的肿瘤或肿瘤症状无法通过其他方法治疗的情况。
