Combined hepatocellular-cholangiocarcinoma after tetralogy of Fallot repair: a case report and review of literature

Kyohei Yugawa, Kenichi Kohashi, Shinji Itoh, Tomoharu Yoshizumi, Ichiro Sakamoto, Hiroyuki Tsutsui, Masaki Mori, Yoshinao Oda

研究成果: ジャーナルへの寄稿学術誌査読

2 被引用数 (Scopus)


Background: Liver fibrosis and cancer are serious hepatic complications for patients with congenital heart diseases. We present a rare case of combined hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) (cHCC-CCA) after the repair of tetralogy of Fallot (TOF). Case presentation: A 54-year-old Japanese woman had undergone biventricular repair for TOF at 7 years old. She presented with abdominal distension. Abdominal CT revealed ascites and a 90-mm tumor involving the liver's left lobe. Tumor marker values were: alpha-fetoprotein, 16,208 ng/mL and des-gamma-carboxy prothrombin, 33,920 mAU/mL. The preoperative diagnosis was malignant tumor of the liver (e.g., HCC or intrahepatic CCA). We performed a left lobectomy of the liver. Histopathologically, the tumor was composed of two components growing in trabecular and irregular tubular patterns accompanied by a transitional area; the tumor was diagnosed as cHCC-CCA. The non-cancerous area showed fibrous change mainly surrounding a central vein and sinusoid, expanding toward the portal area without inflammation. Conclusions: We provide the details of our patient's cHCC-CCA that developed from fibrous congestive liver associated with right-sided heart failure after TOF repair, diagnosed based on histopathological features. We discuss liver fibrosis as a hepatic complication and a careful follow-up maneuver for improving the outcomes of patients with chronic hepatic congestion.

ジャーナルPathology Research and Practice
出版ステータス出版済み - 5月 2020

!!!All Science Journal Classification (ASJC) codes

  • 病理学および法医学
  • 細胞生物学


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