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青少年胆管梗阻的罕见病因:神经内分泌肿瘤。

A rare cause of bile duct obstruction in adolescence: Neuroendocrine tumor.

作者信息

Yalav Orçun, Ülkü Abdullah, Demiryürek Haluk, Doran Figen

机构信息

Department of General Surgery, Çukurova University Faculty of Medicine, Adana, Turkey.

出版信息

Turk J Gastroenterol. 2014 Dec;25 Suppl 1:311-2. doi: 10.5152/tjg.2014.3565.

Abstract

Extrahepatic bile duct is one of the rare places for neuroendocrine tumors which comprise 0.2-2% off all neuroendocrine tumors of gastrointestinal tract. The aim of this paper is to report a case of a 16-year-old boy with a neuroendocrine tumor of extra hepatic bile duct. Laboratory and imaging findings is supported obstructive jaundice. After a pre-operative detailed evaluation, the common bile and common hepatic duct with gallbladder were resected and Roux-en-Y hepaticojejunostomy was performed. After a 36 months follow up, no locoregional recurrence or metastatic disease was observed. Pre-operative diagnosis of neuroendocrine tumors are extremely difficult and often confused with cholangiocarcinoma. Treatment modality of this rare entity depends on the location of the tumor and aggressive surgery is still the best chocie of treatment. It should be kept in mind that disease-free survival for patients with neuroendocrine tumor depends on success of surgery.

摘要

肝外胆管是神经内分泌肿瘤的罕见发病部位之一,在所有胃肠道神经内分泌肿瘤中占0.2%-2%。本文旨在报告一例16岁男孩的肝外胆管神经内分泌肿瘤病例。实验室和影像学检查结果支持梗阻性黄疸。经过术前详细评估,切除了胆总管、肝总管和胆囊,并进行了Roux-en-Y肝空肠吻合术。经过36个月的随访,未观察到局部复发或转移性疾病。神经内分泌肿瘤的术前诊断极其困难,常与胆管癌混淆。这种罕见实体的治疗方式取决于肿瘤的位置,积极的手术仍然是最佳的治疗选择。应牢记,神经内分泌肿瘤患者的无病生存期取决于手术的成功与否。

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