Hepatocellular Carcinoma in a Patient with Hereditary Hemorrhagic Telangiectasia

Kazuhiko Morii; Riku Uematsu; Takeharu Yamamoto; Shinichiro Nakamura; Hiroaki Okushin; Noriyuki Nishiwaki; Takanori Watanabe; Kyohei Kai; Shiso Sato

doi:10.2169/internalmedicine.1056-18

CASE REPORTS

Hepatocellular Carcinoma in a Patient with Hereditary Hemorrhagic Telangiectasia

Kazuhiko Morii, Riku Uematsu, Takeharu Yamamoto, Shinichiro Nakamura, Hiroaki Okushin, Noriyuki Nishiwaki, Takanori Watanabe, Kyohei Kai, Shiso Sato

Author information

Kazuhiko Morii
Department of Hepatology, Japanese Red Cross Society Himeji Hospital, Japan
Riku Uematsu
Department of Hepatology, Japanese Red Cross Society Himeji Hospital, Japan
Takeharu Yamamoto
Department of Hepatology, Japanese Red Cross Society Himeji Hospital, Japan
Shinichiro Nakamura
Department of Hepatology, Japanese Red Cross Society Himeji Hospital, Japan
Hiroaki Okushin
Department of Hepatology, Japanese Red Cross Society Himeji Hospital, Japan
Noriyuki Nishiwaki
Department of Hepatobiliary and Pancreatic Surgery, Shizuoka Cancer Center, Japan
Takanori Watanabe
Department of Hepatobiliary and Pancreatic Surgery, Japanese Red Cross Society Himeji Hospital, Japan
Kyohei Kai
Department of Hepatobiliary and Pancreatic Surgery, Japanese Red Cross Society Himeji Hospital, Japan
Shiso Sato
Department of Hepatobiliary and Pancreatic Surgery, Japanese Red Cross Society Himeji Hospital, Japan

Keywords: hepatocellular carcinoma, hereditary hemorrhagic telangiectasia, vascular malformation, focal nodular hyperplasia, ischemic liver cirrhosis theory

JOURNAL OPEN ACCESS

2018 Volume 57 Issue 24 Pages 3545-3549

DOI https://doi.org/10.2169/internalmedicine.1056-18

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Abstract

A 76-year-old woman with hereditary hemorrhagic telangiectasia (HHT) showed elevated serum hepatobiliary enzyme levels, and abdominal imaging studies revealed a hepatic tumor. Her serum alpha-fetoprotein level was 759.5 ng/mL. A pathological examination after hepatectomy confirmed a diagnosis of hepatocellular carcinoma (HCC). An examination of the surrounding liver revealed dilated vessels and thickened endothelial cells without inflammations. HHT patients without other risk factors (like this patient) reportedly have a lower incidence of common cancers, including HCC, in comparison to the unaffected population. One intriguing hypothesis that might explain the hepatocarcinogenesis in this situation is the ischemic liver cirrhosis theory, which suggests that chronic ischemia may cause parenchymal strain and promote inappropriate hepatocyte proliferation.

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