Zhang Hai-Wen, Kou Kai, Qi Jun, Xie En-Bo, Wang Meng, Li Yan, Lv Guo-Yue, Wang Guang-Yi
1 Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun, Jilin, China.
2 Department of Surgery, School of Medicine, University of Louisville, Louisville, KY, USA.
J Int Med Res. 2019 Jul;47(7):3421-3426. doi: 10.1177/0300060519850391. Epub 2019 Jun 3.
The concept of mixed adenoneuroendocrine carcinoma (MANEC) was introduced in the 2010 World Health Organization classification of digestive neuroendocrine neoplasms. Bile duct invasion by MANEC is exceptionally rare. We herein report a case of MANEC with invasion of multiple bile ducts. Case presentation: A 60-year-old man presented with a 7-day history of upper abdominal pain, and a mass in the cystic duct was suspected based on computed tomography findings. The patient underwent resection of the extrahepatic bile ducts with concomitant radical lymphadenectomy and Roux-en-Y cholangiojejunostomy. Large cell neuroendocrine carcinoma was detected in a component of the resected tumor. According to the pathological and immunohistochemical features of the tumor, the final histopathological diagnosis was a biliary MANEC, tumor stage T2N0M1 (Stage IIIC). The patient recovered uneventfully and was discharged from the hospital 10 days after surgery.
We have described a rare case of extrahepatic MANEC invading multiple bile ducts, with particular emphasis on the physician’s awareness of MANEC and its optimal treatment. MANEC arising from extrahepatic bile ducts is rare, and surgical resection is the most effective treatment method.
混合性腺神经内分泌癌（MANEC）的概念在2010年世界卫生组织消化系统神经内分泌肿瘤分类中被提出。MANEC侵犯胆管极为罕见。我们在此报告一例侵犯多条胆管的MANEC病例。病例介绍：一名60岁男性，有7天的上腹部疼痛病史，根据计算机断层扫描结果怀疑胆囊管有肿块。患者接受了肝外胆管切除并同时进行根治性淋巴结清扫和Roux-en-Y胆管空肠吻合术。在切除的肿瘤成分中检测到了大细胞神经内分泌癌。根据肿瘤的病理和免疫组化特征，最终组织病理学诊断为胆管MANEC，肿瘤分期为T2N0M1（IIIC期）。患者恢复顺利，术后10天出院。
我们描述了一例罕见的肝外MANEC侵犯多条胆管的病例，特别强调了医生对MANEC及其最佳治疗方法的认识。肝外胆管起源的MANEC罕见，手术切除是最有效的治疗方法。
