肝内胆管癌（ICC）是肝脏内一组异质性恶性肿瘤。我们试图根据肿瘤的解剖学起源对 ICC 进行亚型分类，并建议对当前的分类系统进行修改。接受 ICC 根治性切除术、肝门部胆管癌 (CCA) 或肝细胞癌 (HCC) 的患者是从三个国际多中心中确定的。 - 数据库机构联盟。评估临床病理特征和生存结果。在 1264 例 ICC 患者中，1066 例（84.3%）被归类为 ICC 周围亚型，而 198 例（15.7%）被归类为 ICC 肺门周围亚型。与 ICC 周围亚型相比，ICC 肺门周围亚型更常与侵袭性肿瘤特征相关，包括更高的淋巴结转移发生率、大血管和微血管侵犯、神经周围侵犯以及较差的总生存期 (OS)（中位数：ICC -肺门周围 19.8 个月 vs. ICC 外周 37.1 个月；p < 0.001）和无病生存 (DFS)（中位数：ICC-肺门 12.8 vs. ICC 外周 15.2 个月；p = 0.019）。 ICC 肺门周围亚型和肺门 CCA 具有相似的 OS（19.8 个月与 21.4 个月；p = 0.581）和 DFS（12.8 个月与 16.8 个月；p = 0.140）。 ICC 外周亚型肿瘤与更晚期的肿瘤特征以及与 HCC 相比更差的生存结果相关（OS，中位：ICC 外周 37.1 个月 vs HCC 74.3 个月；p< 0.001；DFS，中位：ICC 外周 15.2 个月 vs 74.3 个月；DFS，中位：ICC 外周 15.2 个月 vs 74.3 个月。 HCC 45.5 个月；p< 0.001）。根据不同的临床病理特征和生存结果，ICC 应分为 ICC-肺门和 ICC-外周亚型。 ICC 肝门周围亚型的表现更像是胆管癌（即肝门 CCA），而 ICC 周围亚型的特征和预后更类似于原发性肝脏恶性肿瘤。© 2023。外科肿瘤学会。

Intrahepatic cholangiocarcinoma (ICC) constitutes a group of heterogeneous malignancies within the liver. We sought to subtype ICC based on anatomical origin of tumors, as well as propose modifications of the current classification system.Patients undergoing curative-intent resection for ICC, hilar cholangiocarcinoma (CCA), or hepatocellular carcinoma (HCC) were identified from three international multi-institutional consortia of databases. Clinicopathological characteristics and survival outcomes were assessed.Among 1264 patients with ICC, 1066 (84.3%) were classified as ICC-peripheral subtype, whereas 198 (15.7%) were categorized as ICC-perihilar subtype. Compared with ICC-peripheral subtype, ICC-perihilar subtype was more often associated with aggressive tumor characteristics, including a higher incidence of nodal metastasis, macro- and microvascular invasion, perineural invasion, as well as worse overall survival (OS) (median: ICC-perihilar 19.8 vs. ICC-peripheral 37.1 months; p < 0.001) and disease-free survival (DFS) (median: ICC-perihilar 12.8 vs. ICC-peripheral 15.2 months; p = 0.019). ICC-perihilar subtype and hilar CCA had comparable OS (19.8 vs. 21.4 months; p = 0.581) and DFS (12.8 vs. 16.8 months; p = 0.140). ICC-peripheral subtype tumors were associated with more advanced tumor features, as well as worse survival outcomes versus HCC (OS, median: ICC-peripheral 37.1 vs. HCC 74.3 months; p < 0.001; DFS, median: ICC-peripheral 15.2 vs. HCC 45.5 months; p < 0.001).ICC should be classified as ICC-perihilar and ICC-peripheral subtype based on distinct clinicopathological features and survival outcomes. ICC-perihilar subtype behaved more like carcinoma of the bile duct (i.e., hilar CCA), whereas ICC-peripheral subtype had features and a prognosis more akin to a primary liver malignancy.© 2023. Society of Surgical Oncology.