急性進行性肝血管肉腫。剖検症例報告

Acute progressing hepatic angiosarcoma: An autopsy case report.

• Masaki Inoue
• Mio Matsumoto
• Yusuke Sakuhara
• Yasunari Takakuwa
• Shinji Yoshii
• Nobuaki Akakura
• Naoya Sakamoto

抄録

50歳代の男性が両下肢の浮腫と腹部膨満感を主訴に来院した。病歴と家族歴は、1日66gのアルコール摂取とヘビースモーカーであることを除けば、特に問題はなかった。入院時の血液検査では，軽度の肝機能障害，血小板減少，黄疸，低アルブミン血症，凝固能低下を認めた．腫瘍マーカー検査ではCA19-9とPIVKA-IIの高値を示した。造影CT検査では、初期には主に肝臓の右葉に多発性の腫瘤が増強し、その後、遅発期には肝臓全体にびまん性の増強がみられた。肝動脈造影では，CTで明らかになった腫瘤に対応する大きな血管腫様の病変が認められた．この所見は綿毛のようであった。磁気共鳴画像では、T1強調画像では均一または不均一な低信号強度を示し、T2強調画像では明らかに高信号強度を示す複数の腫瘤様病変が認められた。所見は非定型であり、確定診断はできなかった。肝不全はその後急速に悪化し、20日目に死亡した。剖検の結果、肝血管肉腫と診断された。

A man in his 50s presented with pitting edema of both lower legs and abdominal distension as his chief complaint. His personal medical history and family history were unremarkable, except that he was a heavy drinker consuming 66 g of alcohol per day and a heavy smoker. Blood tests upon admission showed slight hepatic dysfunction, thrombocytopenia, jaundice, hypoalbuminemia, and decreased coagulability. Tumor marker tests showed elevated levels of CA19-9 and PIVKA-II. Contrast-enhanced computed tomography revealed enhancement of multiple masses predominantly in the right lobe of the liver in the early phase, followed by diffuse enhancement of the entire liver in the delayed phase. Hepatic arteriography demonstrated large hemangioma-like lesions corresponding to the masses revealed by computed tomography. That findings seemed to be cotton wool appearance. On magnetic resonance images, there were multiple mass-like lesions that showed homogeneous or heterogeneous low signal intensity on T1-weighted images, and clearly high signal intensity on T2-weighted images. The findings were atypical and no definite diagnosis could be made. Hepatic failure then rapidly worsened, and the patient died on hospital day 20. Autopsy led to the diagnosis of hepatic angiosarcoma.

© 2020 The Authors. Published by Elsevier Inc. on behalf of University of Washington.