Norton J A, Doppman J L, Jensen R T
Surgery Branch, National Cancer Institute, Bethesda, Maryland 20892.
Ann Surg. 1992 Jan;215(1):8-18. doi: 10.1097/00000658-199201000-00012.
Since 1980, 73 patients with Zollinger-Ellison syndrome (ZES) without radiographic evidence of liver metastases were studied on a prospective protocol including medical management of gastric acid hypersecretion, extensive radiographic tumor localization, and exploratory surgery to find and resect gastrinoma for potential cure. Each patient had gastric acid hypersecretion effectively controlled with either H2-blockers or omeprazole. Patients were divided prospectively into two groups, with all patients undergoing the same preoperative localization studies and extensive laparotomy. In contrast to group 1 (1980-1986) (36 patients), group 2 (1987-Oct. 1990) (37 patients) also underwent additional procedures (transillumination and duodenotomy) at surgery to find duodenal gastrinomas. Preoperative imaging studies localized tumor in 38 (52%) patients, and portal venous sampling for gastrin determinations was positive in 49 (67%) patients. Gastrinomas were found and resected in 57 (78%) patients. Significantly more gastrinomas (92% of patients) were found in group 2 than in group 1 patients (64%) (p less than 0.01). This increase was due to increased numbers of duodenal gastrinomas in group 2 than in group 1 patients (43% versus 11%; p less than 0.01). The increased ability to find duodenal gastrinomas did not significantly improve the immediate disease-free rate, which was 58% for all patients. Duodenal primary gastrinomas were found to have a significantly greater incidence of metastases (55%) and a significantly shorter disease-free interval (12 months) than pancreatic gastrinomas (22% and 84 months, respectively) suggesting that duodenal gastrinomas may be more malignant and not more frequently curable than pancreatic gastrinomas. Operations were performed with no deaths and 11% morbidity rate. Long-term follow-up showed that 50% of patients initially rendered disease free would develop recurrent disease by 5 years. Survival was excellent for all patients, and none died of malignant spread of the tumor or uncontrolled peptic ulcer disease, with a mean follow-up of 5 years. This finding is in contrast to patients who presented with metastatic disease on imaging studies and had a 20% 5-year survival rate. This study suggests that all patients with localized sporadic ZES can have the gastric acid hypersecretion managed medically, that overall survival of these patients is excellent, most (78%) can have all gastrinoma found and resected, and some (30%) will be cured (long-term disease-free survival).
自1980年以来，对73例无肝脏转移影像学证据的佐林格-埃利森综合征（ZES）患者进行了一项前瞻性研究，该研究方案包括胃酸分泌过多的药物治疗、广泛的影像学肿瘤定位以及探索性手术，以寻找并切除胃泌素瘤以期治愈。每位患者的胃酸分泌过多均通过H2受体阻滞剂或奥美拉唑得到有效控制。患者被前瞻性地分为两组，所有患者均接受相同的术前定位检查和广泛的剖腹手术。与第1组（1980 - 1986年）（36例患者）相比，第2组（1987年 - 1990年10月）（37例患者）在手术中还进行了额外的操作（透照和十二指肠切开术）以寻找十二指肠胃泌素瘤。术前影像学检查在38例（52%）患者中定位到肿瘤，门静脉采血测定胃泌素在49例（67%）患者中呈阳性。在57例（78%）患者中发现并切除了胃泌素瘤。第2组中发现的胃泌素瘤患者比例（92%）显著高于第1组患者（64%）（p < 0.01）。这种增加是由于第2组中十二指肠胃泌素瘤的数量比第1组患者多（43%对11%；p < 0.01）。发现十二指肠胃泌素瘤能力的提高并未显著改善即时无病率，所有患者的即时无病率为58%。发现十二指肠原发性胃泌素瘤的转移发生率（55%）显著高于胰腺胃泌素瘤（22%），且无病间期显著短于胰腺胃泌素瘤（分别为12个月和84个月），这表明十二指肠胃泌素瘤可能比胰腺胃泌素瘤更具恶性，且治愈的可能性并不更高。手术无死亡病例，发病率为11%。长期随访显示，最初达到无病状态的患者中有50%在5年内会出现疾病复发。所有患者的生存率都很好，平均随访5年，无患者死于肿瘤的恶性扩散或无法控制的消化性溃疡疾病。这一发现与影像学检查显示有转移疾病的患者形成对比，后者的5年生存率为20%。这项研究表明，所有局限性散发性ZES患者的胃酸分泌过多都可以通过药物治疗，这些患者的总体生存率很好，大多数（78%）患者的所有胃泌素瘤都能被发现并切除，一些（30%）患者将被治愈（长期无病生存）。
