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Contemporary surgical management of the Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1.
Best Practice & Research Clinical Endocrinology & Metabolism ( IF 7.4 ) Pub Date : 2019-08-31 , DOI: 10.1016/j.beem.2019.101318
Max B Albers 1 , Jerena Manoharan 1 , Detlef K Bartsch 1
Affiliation  

About 30% of patients with MEN1 develop a Zollinger-Ellison syndrome. Meanwhile it is well established that the causative gastrinomas are almost exclusively localized in the duodenum and not in the pancreas, MEN1 gastrinomas occur multicentric and are associated with hyperplastic gastrin cell lesions and tiny gastrin-producing micro tumors in contrast to sporadic duodenal gastrinomas. Regardless of the high prevalence of early lymphatic metastases, the survival is generally good with an aggressive course of disease in only about 20% of patients. Symptoms can be controlled medically. The indication, timing, type, and extent of surgery are highly controversial and are discussed in detail in this article by a thorough and critical review of literature. More radical procedures, like partial pancreaticoduodenectomy, are weighed against less aggressive local excision of gastrinomas and the pros and cons of both approaches are discussed in terms of long-term morbidity, biochemical cure, and survival.

中文翻译:

多发性内分泌肿瘤1型Zollinger-Ellison综合征的当代外科治疗

大约30%的MEN1患者发展为Zollinger-Ellison综合征。同时,众所周知的是,致病性胃泌素瘤几乎只局限在十二指肠而不是胰腺,MEN1胃泌素瘤多发,与增生性胃泌素细胞病变和微小的胃泌素产生微瘤相关,而散发的十二指肠胃泌素瘤则相反。不管早期淋巴转移的高发率如何,通常只有约20%的患者在侵略性病程中生存良好。症状可在医学上控制。手术的适应症,手术时机,类型和程度存在很大争议,本文将通过对文献进行彻底而严格的评论对其进行详细讨论。更彻底的手术,例如部分胰十二指肠切除术,
更新日期:2019-08-31
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