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

Authors
  • Ishikawa, Yuki1
  • Saito, Ryusuke1
  • Murakami, Keigo2
  • Fujio, Atsushi1
  • Miyazawa, Koji1
  • Sasaki, Kengo1
  • Matsumura, Muneyuki1
  • Mitsugashira, Hiroaki1
  • Degawa, Kazuki1
  • Kobayashi, Yoshinobu3
  • Muto, Ryo3
  • Tokodai, Kazuaki1
  • Furukawa, Toru2
  • Unno, Michiaki1
  • Kamei, Takashi1
  • 1 Department of Surgery, Tohoku University Graduate School of Medicine.
  • 2 Department of Investigative Pathology, Tohoku University Graduate School of Medicine.
  • 3 Department of Surgery, National Hospital Organization Mito Medical Center.
Type
Published Article
Journal
The Tohoku Journal of Experimental Medicine
Publisher
Tohoku University Medical Press
Publication Date
Dec 16, 2023
Volume
261
Issue
4
Pages
267–272
Identifiers
DOI: 10.1620/tjem.2023.J080
PMID: 37766552
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

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.

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