Department of Analytical Pathology, National Cancer Center Research Institute, Tokyo, Japan.
Hepatobiliary and Pancreatic Surgery Division, National Cancer Center Hospital, Tokyo, Japan.
Pathol Int. 2022 Jun;72(6):332-342. doi: 10.1111/pin.13226. Epub 2022 Apr 26.
Hepatoid carcinoma or related entities (HPC/RTs) are extremely rare, especially in the extrahepatic bile duct (EHBD). Only a few case reports have been published. We analyzed the clinicopathological features of HPCs/RTs in EHBD. HPC/RT of extrahepatic cholangiocarcinoma (eCCA) cases were selected based on the histological characteristics and immunohistochemical detection of spalt-like transcription factor 4 (SALL4) and/or alpha-fetoprotein (AFP). Four HPC/RT cases arose in the distal but not in the perihilar EHBD. The four patients with HPC/RT included one female and three males with a median age of 77 years. There are various macroscopic types of HPC/RT. The predominant histological features were two solid-type carcinomas that mimicked hepatocellular carcinoma and two well-differentiated tubular adenocarcinomas. Immunohistochemically, SALL4 and glypican-3 were expressed in all cases, and AFP was expressed in one case. Cancer cell phenotypes included intestinal, pancreatobiliary, and mixed pancreatobiliary and intestinal types. Focal neuroendocrine differentiation and severe perineural and lymphovascular invasions were also observed. HPC/RT recurred in two patients within 2 years, and one patient died 13 months postoperatively. It is suggested that the HPC/RT of EHBD shares common characteristics with HPC/RT arising in various organs, and has some unique characteristics. HPC/RT of EHBD might be more aggressive than conventional eCCA.
肝样细胞癌或相关肿瘤（HPC/RTs）非常罕见，尤其是在外胆管（EHBD）中。仅有少数病例报告发表。我们分析了 EHBD 中 HPCs/RTs 的临床病理特征。根据 HPC/RT 的组织学特征和 SALL4 和/或 AFP 的免疫组织化学检测，选择肝外胆管癌（eCCA）病例中的 HPC/RT。4 例 HPC/RT 发生在远端 EHBD，而非肝门部。4 例 HPC/RT 患者包括 1 名女性和 3 名男性，中位年龄为 77 岁。HPC/RT 有多种大体类型。主要的组织学特征是两种类似于肝细胞癌的实体型癌和两种分化良好的管状腺癌。免疫组化检查，所有病例均表达 SALL4 和 Glypican-3，1 例表达 AFP。癌细胞表型包括肠型、胰胆管型和混合胰胆管型。还观察到局灶性神经内分泌分化和严重的神经周围和血管侵犯。2 例患者在 2 年内复发，1 例患者术后 13 个月死亡。提示 EHBD 的 HPC/RT 与发生于各种器官的 HPC/RT 具有共同特征，并有一些独特特征。EHBD 的 HPC/RT 可能比常规 eCCA 更具侵袭性。
