Thompson J C, Reeder D D, Villar H V, Fender H R
Surg Gynecol Obstet. 1975 May;140(5):721-39.
With better methods of diagnosis, patients will be identified earlier in the course of their disease and will often have atypical and borderline manifestations of the syndrome. Serum gastrin measurements with calcium and especially with secretin challenge will be the most important method of diagnosis. Any patient with acid hypersecretion who has a high serum gastrin level that does higher on secretin infusion should be considered to have the Zollinger-Ellison syndrome. A firm diagnosis of the Zollinger-Ellison syndrome should be made, if at all possible, prior to operation. At operation, a thorough search of the pancreas, duodenum, stomach, greater and lesser omentum and liver should be made for primary and secondary gastrinomas. If the preoperative data firmly establish the diagnosis of the Zollinger-Ellison syndrome, a total gastrectomy should be carried out even if no primary tumor is found. Similarly, a total gastrectomy should be done even if there are massive hepatic metastases. If total gastrectomy is not performed, the patient is apt to die of complications of acid hypersecretion. The only possible exceptions to the rule of always performing a total gastrectomy are in asymptomatic patients with easily excisable tumors or patients with tumors of the duodenum that are easily excisable, providing that in both instances after the excision of the tumor the output of gastric acid as measured at operation is immediately halted. All possible metastatic tumor tissue should be removed. The more tumor tissue removed, the longer the patient will survive. Metastases should be treated aggressively. They do not disappear after total gastrectomy in our experience, and they may kill patients. Patients should be followed after operation with serial measurements of serum gastrin concentrations and by hepatic scintillation scans and hepatic angiography. If hepatic metastases develop, intrahepatic artery infusions of 5-fluorouracil may slow tumor growth.
随着诊断方法的改进，患者在疾病过程中能够被更早地识别出来，且往往会出现该综合征的非典型和临界表现。血清胃泌素测定，尤其是钙刺激试验和促胰液素激发试验，将成为最重要的诊断方法。任何胃酸分泌过多且血清胃泌素水平高，在注射促胰液素后升高的患者，都应被视为患有佐林格 - 埃利森综合征。如果可能的话，应在手术前对佐林格 - 埃利森综合征做出明确诊断。手术时，应对胰腺、十二指肠、胃、大网膜和小网膜以及肝脏进行全面探查，以寻找原发性和继发性胃泌素瘤。如果术前数据能明确诊断佐林格 - 埃利森综合征，即使未发现原发性肿瘤，也应进行全胃切除术。同样，即使存在大量肝转移，也应进行全胃切除术。如果不进行全胃切除术，患者容易死于胃酸分泌过多的并发症。全胃切除术这一规则唯一可能的例外情况是，对于无症状且肿瘤易于切除的患者，或十二指肠肿瘤易于切除的患者，前提是在这两种情况下，肿瘤切除后手术时测得的胃酸分泌立即停止。应切除所有可能的转移瘤组织。切除的肿瘤组织越多，患者存活的时间就越长。应积极治疗转移瘤。根据我们的经验，全胃切除术后转移瘤不会消失，且可能导致患者死亡。术后应通过连续测量血清胃泌素浓度、肝脏闪烁扫描和肝血管造影对患者进行随访。如果发生肝转移，肝动脉内注入5 - 氟尿嘧啶可能会减缓肿瘤生长。
