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Biliary Atresia and Malignancy

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Introduction to Biliary Atresia

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Abstract

Liver fibrosis caused by cholestasis progresses even after the Kasai portoenterostomy in biliary atresia. The development of a malignant tumor is one of the late complications of biliary atresia.

Hepatocellular carcinoma is the most common malignant tumor. Before the era of the Kasai portoenterostomy, three cases of hepatocellular carcinoma in the postmortem examination had been reported since 1965.

In the explanted liver at the time of transplant surgery, hepatocellular carcinomas have been frequently reported since 1994. All the patients notably have been alive since liver transplantation. During the follow-up period after portoenterostomy, on the other hand, a total of 18 patients were clinically diagnosed as having hepatocellular carcinoma, of whom eleven patients were diagnosed before the teenage. The age at the Kasai portoenterostomy and outcome of jaundice were not associated with malignant development. However, at more than 16 years of age, four out of seven patients underwent alternative treatments other than liver transplant surgery, suggesting that older patients may have a progressive change of hepatocellular carcinoma development in the cirrhotic liver. Posttransplant prognosis is relatively good in patients who are early diagnosed during the follow-up period after the Kasai portoenterostomy.

Cholangiocarcinomas were found in seven biliary atresia patients, older than those of hepatocellular carcinoma. The prognosis of cholangiocarcinoma is worse than that of hepatocellular carcinoma. Six patients died, despite aggressive adjuvant chemotherapy for recurrent and metastatic disease.

Hepatoblastomas were found in four biliary atresia patients before five years of age. The relationship remains unknown between hepatoblastoma and biliary atresia. Hepatoblastoma was found in the explanted liver on the liver transplantation in two patients and found clinically during the follow-up period in the rest two patients. Three patients underwent liver transplantation with good results.

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Authors and Affiliations

  1. Department of Pediatric Surgery, Nihon University School of Medicine, Tokyo, Japan

    Tsugumichi Koshinaga

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Correspondence to Tsugumichi Koshinaga .

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  1. Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan

    Masaki Nio

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Koshinaga, T. (2021). Biliary Atresia and Malignancy. In: Nio, M. (eds) Introduction to Biliary Atresia. Springer, Singapore. https://doi.org/10.1007/978-981-16-2160-4_42

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  • DOI: https://doi.org/10.1007/978-981-16-2160-4_42

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