Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea.
Department of Pathology, Chonnam National University Medical School, Gwangju, Korea.
Korean J Gastroenterol. 2023 Mar 25;81(3):121-124. doi: 10.4166/kjg.2023.005.
Neuroendocrine carcinoma (NEC) arising from the extrahepatic bile duct is extremely rare and commonly mistaken for cholangiocarcinoma. Therefore, NEC of the bile duct is difficult to diagnose preoperatively. Previously reported cases were resected with a diagnosis of cholangiocarcinoma and diagnosed with NEC after surgery. This paper reports an 84-year-old female with small-cell NEC of the extrahepatic bile duct, confirmed by a biopsy from an ERCP, with a review of the relevant literature. Contrast-enhanced abdomen computed tomography and magnetic resonance cholangiopancreatography revealed an approximately 1.7 cm enhancing intraductal mass in the proximal common bile duct with dilatation of the upstream bile duct. ERCP showed a long strictured segment in the proximal common bile duct with bile duct dilatation. A biopsy was performed at the site of the stricture. Histological examinations and hematoxylin-eosin staining showed the solid proliferation of small tumor cells with irregularly shaped hyperchromatic nuclei. Immunohistochemical examinations showed that the tumor cells were positive for CD56 and synaptophysin. Small-cell NEC of the extrahepatic bile duct was confirmed based on the histology and immunohistochemistry findings. The patient and their family denied treatment because of the patient's old age.
发生于肝外胆管的神经内分泌癌（NEC）极其罕见，通常易误诊为胆管癌。因此，胆管 NEC 术前难以诊断。既往报道的病例均在诊断为胆管癌行切除术，术后诊断为 NEC。本文报道了一例经内镜逆行胰胆管造影（ERCP）活检证实的 84 岁女性肝外胆管小细胞 NEC，同时复习相关文献。腹部增强 CT 和磁共振胰胆管成像显示胆总管近端约 1.7cm 强化的腔内肿块，上游胆管扩张。ERCP 显示近端胆总管长段狭窄，胆管扩张。于狭窄处行活检。组织学检查和苏木精-伊红染色显示小肿瘤细胞实性增生，细胞核形状不规则，染色质深染。免疫组织化学检查显示肿瘤细胞 CD56 和突触素阳性。基于组织学和免疫组织化学结果，确诊为肝外胆管小细胞 NEC。因患者高龄，患者及其家属拒绝治疗。
