Ishikawa Yuki, Saito Ryusuke, Murakami Keigo, Fujio Atsushi, Miyazawa Koji, Sasaki Kengo, Matsumura Muneyuki, Mitsugashira Hiroaki, Degawa Kazuki, Kobayashi Yoshinobu, Muto Ryo, Tokodai Kazuaki, Furukawa Toru, Unno Michiaki, Kamei Takashi
Department of Surgery, Tohoku University Graduate School of Medicine.
Department of Investigative Pathology, Tohoku University Graduate School of Medicine.
Tohoku J Exp Med. 2023 Dec 16;261(4):267-272. doi: 10.1620/tjem.2023.J080. Epub 2023 Sep 28.
Biliary atresia is an obliterative cholangiopathy of unknown etiology. Hepatic portoenterostomy, in which obliterated extrahepatic bile ducts are resected and bile flow is restored, known as Kasai operation, is performed within 3 months after birth. While this operation enhances long-term survival of patients, the occurrence of primary malignant hepatic tumors has been increasing. We report a case of small intestinal adenocarcinoma arising at the anastomotic site after Kasai operation. A 49-year-old man, who underwent Kasai operation for biliary atresia when he was 2 months old, experienced rapidly progressive jaundice and liver dysfunction. Deceased-donor liver transplantation was performed for liver failure. Macroscopically, there was a white-yellow tumor located at the anastomotic site of hepatic portoenterostomy of the resected liver. Pathological examination revealed a well-differentiated adenocarcinoma with some Paneth cells in the neoplastic lesion. Immunohistochemically, the tumor cells were negative for cytokeratin 7 (CK7) but positive for cytokeratin 20 (CK20) and a homeobox domain-containing transcription factor (CDX2). Mucin expression in tumor cells was negative for mucin 1 (MUC1) and mucin 6 (MUC6) and positive for mucin 2 (MUC2) and mucin 5AC (MUC5AC). The pathological diagnosis was small intestinal adenocarcinoma originating from the jejunum. The patient was discharged 48 days after the operation. The patient had not experienced recurrence at 10 months after the operation. This is the first report of small intestinal adenocarcinoma arising at the anastomotic site after Kasai operation for biliary atresia. Special care should be taken for the patients after Kasai operation with acute progressive jaundice and liver dysfunction because there is a possibility of malignancy in their native liver.
胆道闭锁是一种病因不明的闭塞性胆管病。肝门肠吻合术，即切除闭塞的肝外胆管并恢复胆汁流动，也就是所谓的葛西手术，在出生后3个月内进行。虽然这种手术提高了患者的长期生存率，但原发性恶性肝肿瘤的发生率一直在上升。我们报告一例葛西手术后吻合口处发生小肠腺癌的病例。一名49岁男性，2个月大时因胆道闭锁接受了葛西手术，之后出现快速进展的黄疸和肝功能障碍。因肝衰竭进行了尸肝移植。大体检查显示，在切除肝脏的肝门肠吻合术吻合口处有一个黄白色肿瘤。病理检查显示为高分化腺癌，肿瘤病变中有一些潘氏细胞。免疫组化显示，肿瘤细胞细胞角蛋白7（CK7）阴性，但细胞角蛋白20（CK20）和含同源框结构域的转录因子（CDX2）阳性。肿瘤细胞中的黏蛋白表达，黏蛋白1（MUC1）和黏蛋白6（MUC6）阴性，黏蛋白2（MUC2）和黏蛋白5AC（MUC5AC）阳性。病理诊断为起源于空肠的小肠腺癌。患者术后48天出院。术后10个月未出现复发。这是首例关于胆道闭锁葛西手术后吻合口处发生小肠腺癌的报告。对于葛西手术后出现急性进行性黄疸和肝功能障碍的患者应特别关注，因为其原生肝脏有发生恶性病变的可能。
