Meko J B, Norton J A
Department of General Surgery, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
Annu Rev Med. 1995;46:395-411. doi: 10.1146/annurev.med.46.1.395.
Zollinger-Ellison syndrome (ZES) is caused by gastrin-secreting tumors called gastrinomas. Patients commonly present with peptic ulcer disease and may have recurrent, multiple, and atypically located ulcers, e.g. in the jejunum. Alternatively, severe diarrhea may be the only presenting symptom. Patients with multiple endocrine neoplasia Type I (MEN-I) and ZES become symptomatic at an earlier age than patients with sporadic ZES. Patients with ZES have elevated fasting serum gastrin concentrations (> 100 pg/ml) and basal gastric acid hypersecretion (> 15 mEq/h). The secretin stimulation test is the best test to distinguish ZES from other conditions resulting in elevated gastrin levels. Gastric acid hypersecretion can be controlled in virtually all patients with H2-receptor antagonists or omeprazole, thus rendering total gastrectomy unnecessary. Computed tomography (CT), magnetic resonance imaging (MRI), radionuclide octreotide scanning, endoscopic ultrasound, and the selective arterial secretin injection test are the recommended imaging studies for localization of gastrinoma; nevertheless, 50% of gastrinomas are not evident on preoperative imaging studies. All patients with sporadic gastrinoma who do not have unresectable metastatic disease should undergo exploratory laparotomy for potential curative resection. With increased awareness of duodenal tumors, gastrinoma can be found in 80-90% of patients. Surgery may be the most effective treatment for metastatic gastrinoma if most or all of the tumor can be resected. The management of patients with MEN-I and ZES remains controversial. Some clinicians advocate an aggressive surgical approach, whereas others have had little success in rendering patients eugastrinemic.
卓-艾综合征（ZES）由称为胃泌素瘤的胃泌素分泌肿瘤引起。患者通常表现为消化性溃疡病，可能有复发性、多发性且位置不典型的溃疡，如空肠溃疡。或者，严重腹泻可能是唯一的首发症状。与散发性ZES患者相比，I型多发性内分泌腺瘤病（MEN-I）合并ZES的患者出现症状的年龄更早。ZES患者空腹血清胃泌素浓度升高（>100 pg/ml）且基础胃酸分泌过多（>15 mEq/h）。促胰液素刺激试验是区分ZES与其他导致胃泌素水平升高的疾病的最佳检查。几乎所有患者使用H2受体拮抗剂或奥美拉唑都能控制胃酸分泌过多，因此无需进行全胃切除术。计算机断层扫描（CT）、磁共振成像（MRI）、放射性核素奥曲肽扫描、内镜超声和选择性动脉注射促胰液素试验是推荐用于胃泌素瘤定位的影像学检查；然而，50%的胃泌素瘤在术前影像学检查中不明显。所有无不可切除转移性疾病的散发性胃泌素瘤患者均应接受剖腹探查术，以进行可能的根治性切除。随着对十二指肠肿瘤认识的提高，80% - 90%的患者可发现胃泌素瘤。如果大部分或全部肿瘤能够切除，手术可能是转移性胃泌素瘤最有效的治疗方法。MEN-I合并ZES患者的管理仍存在争议。一些临床医生主张积极的手术方法，而另一些医生在使患者胃泌素水平正常化方面收效甚微。
