Curative resection in Zollinger-Ellison syndrome. Results of a 10-year prospective study.
Curative resection in Zollinger-Ellison syndrome. Results of a 10-year prospective study.
复制标题
佐林格-埃利森综合征的根治性切除。
作者：
J. Norton;J. Doppman;R. Jensen
自1980年以来，对73例无肝转移影像学证据的Zollinger-Ellison综合征（ZES）患者进行了前瞻性研究，包括胃酸分泌过多的药物治疗，广泛的影像学肿瘤定位，以及探查性手术以发现和切除胃泌素瘤以获得潜在的治愈。每例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。患者前瞻性分为两组，所有患者均接受相同的术前定位研究和广泛的剖腹手术。与第1组（1980-1986年）（36例患者）相比，第2组（1987- 1990年10月）（37例患者）在手术时还接受了额外的手术（透照和十二指肠切开术）以发现十二指肠胃泌素瘤。术前影像学检查定位肿瘤38例（52%），门静脉采样胃泌素测定阳性49例（67%）。57例（78%）患者发现并切除了胃癌。第2组中发现的胃泌素瘤（92%的患者）显著多于第1组患者（64%）（p <0.01）。这一增加是由于第2组患者的十二指肠胃泌素瘤数量比第1组患者增加（43% vs 11%; p <0.01）。发现十二指肠胃泌素瘤的能力的提高并没有显著提高即刻无病率，所有患者的无病率为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（分别为22%和84个月），无病间期（12个月）显著短于胰腺胃泌素瘤（分别为22%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恶性，且不常治愈。无手术死亡，并发症发生率为11%。长期随访显示，50%的患者最初无病，5年后复发。所有患者的生存率都很好，没有人死于肿瘤的恶性扩散或不受控制的消化性溃疡疾病，平均随访5年。这一发现与在影像学检查中表现为转移性疾病且5年生存率为20%的患者相反。这项研究表明，所有局限性散发性ZES患者都可以通过药物治疗胃酸分泌过多，这些患者的总体生存率很高，大多数（78%）可以发现并切除所有胃泌素瘤，一些（30%）将被治愈（长期无病生存）。
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).