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目的结合文献分析并总结肝外胆管神经内分泌癌的临床病理特点、诊断和治疗。方法回顾性分析我院收治的1例肝外胆管神经内分泌癌的临床病理特征并结合国内外文献进行分析总结。结果本组1例患者为男性,58岁,以右上腹间断胀痛为主诉,影像学检查示胆总管下端占位并胆管梗阻,手术治疗,术后病理诊断:胆总管末端神经内分泌癌。患者术后恢复顺利。半年后复查,肿瘤多发转移,再1个月后患者因肿瘤广泛转移而死亡。查阅国内外文献,共检索到27篇共27例已报道病例,本病临床表现与一般胆管癌相同,主要表现为黄疸和上腹部疼痛不适。治疗以手术治疗为主,部分患者辅以化疗或放疗,术后生存时间1~45个月。结论肝外胆管神经内分泌癌是罕见的胆管恶性肿瘤,临床表现以腹痛、黄疸常见。影像学检查可定位但无法定性,目前仅能通过病理及免疫组织化学确诊。治疗以手术为主,但疗效较差。
Objective To analyze and summarize the clinical and pathological features, diagnosis and treatment of extrahepatic bile duct neuroendocrine carcinoma in combination with the literature. Methods The clinical and pathological features of 1 case of extrahepatic bile duct neuroendocrine carcinoma in our hospital were retrospectively analyzed and analyzed based on the literature both at home and abroad. Results One patient in this group was male, 58 years old. The main complaint was intermittent pain in the right upper abdomen. Radiographic examination showed the lower part of the common bile duct and bile duct obstruction. Surgical treatment was performed. Postoperative pathological diagnosis: neuroendocrine carcinoma of the common bile duct. The patient recovered smoothly. Six months after the review, multiple tumor metastases, another one month after the patient died of extensive metastases. Access to domestic and foreign literature, a total of 27 retrieved 27 cases were reported, the clinical manifestations of this disease and the same general cholangiocarcinoma, mainly for jaundice and upper abdominal pain discomfort. Surgical treatment of the main treatment, some patients supplemented by chemotherapy or radiotherapy, postoperative survival time of 1 to 45 months. Conclusions Extrahepatic bile duct neuroendocrine carcinoma is a rare bile duct malignant tumor. The clinical manifestations are abdominal pain and jaundice. Imaging examination can locate but can not be qualitative, currently only confirmed by pathology and immunohistochemistry. Surgical treatment based, but less effective.