Objective: This study aimed to demonstrate the clinical features and postoperative outcomes of extrahepatic bile duct (EHBD) neuroendocrine carcinoma (NEC) and compared with those of adenocarcinoma. Methods: We retrospectively analyzed patients with EHBD cancer operated in our institution between 1995 and 2015. Results: Of 475 patients, 468 had adenocarcinoma, while 7 had NEC/mixed adenoneuroendocrine carcinoma (MANEC) in this study. There were no notable preoperative and pathological features in patients with NEC/MANEC. However, patients with NEC/MANEC had a higher recurrence rate (51.8 vs. 100%, p = 0.016), poorer relapse-free survival (RFS) time (the median RFS time: 35 vs. 12 months, p = 0.006), and poorer overall survival (OS) time (the median OS time: 60 vs. 19 months, p = 0.078) than those with adenocarcinoma. Furthermore, patients with NEC/MANEC had higher rates of liver metastasis (11.9 vs. 85.7%, p < 0.001) than those with adenocarcinoma. In multivariable regression analysis, pathological type with NEC/MANEC was a risk factor for poorer RFS (p = 0.022, hazard ratio: 6.09). Conclusions: Patients with NEC/MANEC have high malignant potential and poor outcomes. It is necessary to develop an effective approach and postoperative adjuvant treatment for patients with NEC/MANEC.

中文翻译：

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胆管神经内分泌癌与腺癌临床病理特征比较

目的：本研究旨在展示肝外胆管（EHBD）神经内分泌癌（NEC）的临床特征和术后结果，并与腺癌进行比较。方法：我们回顾性分析了 1995 年至 2015 年在我们机构手术的 EHBD 癌症患者。结果：在本研究中，475 名患者中，468 名患有腺癌，7 名患有 NEC/混合腺神经内分泌癌（MANEC）。NEC/MANEC 患者没有明显的术前和病理特征。然而，NEC/MANEC 患者的复发率较高（51.8% 与 100%，p = 0.016），无复发生存 (RFS) 时间较短（中位 RFS 时间：35 个月与 12 个月，p = 0.006），和比腺癌患者更差的总生存 (OS) 时间（中位 OS 时间：60 个月与 19 个月，p = 0.078）。此外，NEC/MANEC 患者的肝转移率高于腺癌患者（11.9% vs. 85.7%，p < 0.001）。在多变量回归分析中，NEC/MANEC 的病理类型是较差 RFS 的危险因素（p = 0.022，风险比：6.09）。结论：NEC/MANEC 患者具有较高的恶性潜能和较差的预后。有必要为 NEC/MANEC 患者制定有效的方法和术后辅助治疗。