Department of Surgery, Columbia University Medical Center, New York, New York, USA; Department of Surgery, Hadassah-Hebrew University Medical Center, Mount Scopus, Jerusalem, Israel.
Oncologist. 2014 Jan;19(1):44-50. doi: 10.1634/theoncologist.2013-0369. Epub 2013 Dec 6.
Zollinger-Ellison syndrome (ZES) is an endocrinopathy characterized by gastrin-secreting tumors, responsible for causing the formation of multiple, refractory, and recurrent peptic ulcers in the distal duodenum and proximal jejunum. Two main variants have been described, sporadic and those found in association with parathyroid and pituitary tumors, a genetic disorder known as multiple endocrine neoplasia-1 (MEN-1). Biochemical serum evaluation for elevated gastrin, followed by radiological or nuclear localization of the primary lesion, is mandated for establishing diagnosis. The mainstays of treatment include management of hypersecretory state with medical suppression of gastric acid production and surgical resection of primary tumor for the prevention of malignant transformation and metastatic complications. Medical therapy with proton pump inhibitors has virtually eliminated the need for acid-reducing surgical procedures. Surgical approach to sporadic and MEN-1-associated ZES varies based on our understanding of the natural history of the condition and the probability of cure; however, resection to a negative microscopic margin is indicated in both cases. Postoperative surveillance involves measurement of gastrin level, followed by imaging if elevation is detected. Re-excision of recurrent or resection of metastatic disease is a subject of controversy; however, at the present time aggressive cytoreductive approach is favored.
卓-艾综合征（ZES）是一种内分泌疾病，其特征为胃泌素分泌性肿瘤，导致远端十二指肠和近端空肠多发、难治性、复发性消化性溃疡。已经描述了两种主要变异型，散发性和与甲状旁腺和垂体肿瘤相关的变异型，这是一种称为多发性内分泌肿瘤 1 型（MEN-1）的遗传疾病。为了确诊，需要进行血清胃泌素升高的生化评估，然后进行原发性病变的放射学或核定位。治疗的主要方法包括抑制胃酸分泌的药物治疗和手术切除原发性肿瘤以预防恶性转化和转移并发症，以控制高分泌状态。质子泵抑制剂的药物治疗几乎消除了降低胃酸的手术需求。散发性和 MEN-1 相关 ZES 的手术方法因对疾病自然史和治愈可能性的理解而异；然而，在两种情况下都需要切除至阴性显微镜下边缘。术后监测包括测量胃泌素水平，如果升高，则进行影像学检查。复发性疾病的再次切除或转移性疾病的切除是一个有争议的问题；然而，目前提倡积极的细胞减灭术。
