Department of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Japan.
Department of Pediatric Surgery, Kanazawa Medical University, Ishikawa, Japan.
Pathol Int. 2020 Nov;70(11):888-892. doi: 10.1111/pin.13016. Epub 2020 Sep 16.
Kasai operation is widely performed for biliary atresia (BA), as it improves the prognosis. Biliary tract cancer has rarely been reported as a complication after Kasai operation. A 17-year-old man underwent liver transplantation for progressive jaundice and liver dysfunction after Kasai operation for BA. A macroscopic examination of the explanted liver revealed a 3.6-cm-diameter mass in the hilus of the explanted liver. The tumor consisted of an atypical proliferation of glandular cells in the collagenous stroma. The differential diagnosis included a florid ductular reaction and primary adenocarcinoma. Immunohistochemistry revealed that the glandular cells were diffusely positive for IGF2BP3 and S100P, but negative for CDX2. A diagnosis of extrahepatic cholangiocarcinoma arising from the liver hilus was made. To our knowledge, this is the third case of perihilar cholangiocarcinoma after Kasai operation for BA. The previously reported cases had a poor prognosis, but the current case did not recur during a 15-year follow-up. Cholangiocarcinoma and hepatocellular carcinoma can occur as a late complication of BA after Kasai operation, and early detection and liver transplantation may improve the prognosis.
胆道闭锁（BA）患者常采用葛西手术治疗，该手术能改善预后。然而，胆道癌在葛西手术后极为罕见。本文报道 1 例 17 岁男性患者，因 BA 行葛西手术后出现进行性黄疸和肝功能障碍，行肝移植治疗。术后肝脏大体检查发现肝门部有 3.6cm 直径的肿块。肿瘤由富含胶原的基质中腺样细胞的不典型增生组成。鉴别诊断包括活跃的胆管反应和原发性腺癌。免疫组化显示，腺体细胞弥漫性表达 IGF2BP3 和 S100P，但 CDX2 阴性。诊断为肝门部胆管细胞癌。据我们所知，这是第 3 例 BA 行葛西手术后发生肝门部胆管癌。既往报道的病例预后不良，但本病例在 15 年随访期间未复发。胆管癌和肝细胞癌可作为 BA 行葛西手术后的晚期并发症发生，早期发现和肝移植可能改善预后。
