Small Intestinal Adenocarcinoma Arising at the Anastomotic Site after Kasai Operation for Biliary Atresia: A Case Report and Literature Review

• Published in: The Tohoku Journal of Experimental Medicine Vol. 261; no. 4; pp. 267 - 272
• Main Authors: 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
• Format: Journal Article
• Language: English
• Published: Japan Tohoku University Medical Press 2023
• Subjects: Adenocarcinoma - diagnosis; biliary atresia; Biliary Atresia - surgery; Humans; Infant; Intestinal Neoplasms - diagnosis; Jaundice; Kasai operation; Liver Diseases; Liver Transplantation; Male; malignant tumor; Middle Aged; small intestinal adenocarcinoma; Treatment Outcome; biliary atresia; small intestinal adenocarcinoma; malignant tumor; liver transplantation; Kasai operation
• ISSN: 0040-8727; 1349-3329
• DOI: 10.1620/tjem.2023.J080

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.