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症例は, 48歳女性。30歳時より原因不明の肝機能異常を指摘されており, 41歳時に針生検にて肝線維症と診断されている。多量の腹水が出現し入院した。CT検査にて, 肝左葉および右葉前区域は低吸収域で萎縮しており, 右葉後区域は肥大し, 両側肺には多発性の小結節を認めた。腹腔動脈造影検査では, 左肝動脈および右肝動脈前区域枝の閉塞を認め, 門脈相では拡張した門脈右葉後区域枝のみが造影された。剖検にて, 肝左葉と右葉前区域に多数の癒合した白色結節を認めた。結節の病理組織所見は, 好酸性の豊かな胞体を有する類上皮様の腫瘍細胞が血管腔内に浸潤していた。免疫染色では, 第VIII因子関連抗原, CD34が陽性, EMA, CAM5.2は陰性であった。電顕検査では, Weibel-Palade body を認めた。以上の病理所見より, 本症例は長年にわたる緩徐な臨床経過の後に, 肝動脈, 門脈, 肝静脈に浸潤し門脈圧亢進症および肝不全を呈した肝原発の類上皮血管内皮腫と診断した。 / Epithelioid hemangioendothelioma is a rare vascular neoplasm arising in various sites. such as soft tissue, the liver and the lungs. Though it is considered a low grade malignant neoplasm, some cases show progressive growth leading to metastasis and death. We present an autopsy case of hepatic epithelioid hemangioendothelioma with portal hypertension and hepatic failure. A 48-year-old female was admitted in October 1996 with the menifestation of large amount of ascites. She had a history of liver dysfunction with unknown cause since the age of 30 years. Needle biopsy specimens at the age of 41 years showed fibrosis of the liver. CT scan showed the atrophy of the left lobe and anterior segment of the right lobe of the liver with low density. A postmortem examination macroscopically showed the atrophic left lobe of the liver due to the many white nodules invading to porta hepatis and the hepatic veins. Microscopically the nodules were made of epithelioid shaped tumor cells with abundant eosinophilic cytoplasm invading into sinusoids and vessels. The endothelial origin of the tumor cells was confirmed by the immunohistochemical stain of the Factor VIII and CD34. Ultrastructually Weibel-Palade body was also revealed. We diagnosed hepatic epithelioid hemangioendothelioma invading to the hepatic artery, the portal vein and the hepatic vein with clinical manifestation of the portal hypertension and hepatic failure after long-term mild course. |
