Mignon M, Benhamou G
Service d'Hépato-gastroentérologie, Hôpital Bichat, Paris, France.
Acta Chir Belg. 1991 Mar-Apr;91(2):88-95.
A diagnostic and therapeutic strategy for ZES is proposed based on the vast experience (180 cases) of this rare disease gained at hospital Bichat (Paris, France). The first step is diagnostic and relies essentially a) upon measurements of gastric acid and serum gastrin in the basal state and b) upon the results of secretin test because of the overlap between ZES and duodenal ulcer disease in a large proportion of cases. In sporadic ZES cases, after adequate control of acid overproduction and attempt to localize the tumoral process(es), surgery is indicated (at the exception of patients with advanced metastatic disease). Surgery aims essentially at eradicating gastrinoma(s) as often as possible and whenever it is feasible without endangering patient's life. An apparently definite cure is attained in 60 to 80% in extrapancreatic gastrinoma(s) and 20 to 30% when gastrinomas are located within the pancreas. Liver involvement (25% of ZES cases) remains the major concern and death cause in these patients. Although liver metastases frequently stabilize and sometimes regress upon chemotherapy and chemoembolization, liver transplantation may, in the future, represent the major chance for these patients; but in this frequently slowly evolving condition, the time for liver transplantation is exceptionally difficult to settle.
基于法国巴黎比沙医院在这种罕见疾病（180例）方面积累的丰富经验，提出了一种卓-艾综合征（ZES）的诊断和治疗策略。第一步是诊断，主要基于：a）基础状态下胃酸和血清胃泌素的测量；b）由于在大部分病例中ZES和十二指肠溃疡病存在重叠，因此基于促胰液素试验的结果。在散发性ZES病例中，在充分控制胃酸分泌过多并尝试定位肿瘤过程后，建议进行手术（晚期转移性疾病患者除外）。手术的主要目的是尽可能多地、在可行且不危及患者生命的情况下根除胃泌素瘤。胰腺外胃泌素瘤患者的明显治愈率为60%至80%，胃泌素瘤位于胰腺内时治愈率为20%至30%。肝脏受累（占ZES病例的25%）仍然是这些患者的主要关注点和死亡原因。尽管肝转移在化疗和化疗栓塞后常趋于稳定，有时甚至会消退，但肝移植在未来可能是这些患者的主要希望；但在这种通常进展缓慢的疾病中，肝移植的时机极难确定。
