Kamijo Yuma, Iwadare Takanobu, Fujita Kaede, Kimura Takefumi, Kunimoto Hideo
Department of Hepatology, Nagano Municipal Hospital, Nagano, Japan.
Department of Medicine, Division of Gastroenterology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto,, Nagano, 390-8621, Japan.
Clin J Gastroenterol. 2025 Jul 14. doi: 10.1007/s12328-025-02170-7.
Hepatic angiosarcoma (HAS) is a rare, highly aggressive vascular malignancy. HAS presents with nonspecific clinical and imaging findings, often leading to a delayed or missed diagnosis. We describe a case of HAS in a 78-year-old woman with the chief complaints of progressive abdominal distension and anorexia. She had no history of drinking or hepatotoxic medications. Serologic testing excluded viral and autoimmune hepatitis. Contrast-enhanced computed tomography showed no discrete hepatic mass but revealed diffusely heterogeneous low-attenuation areas with patchy arterial enhancement, marked ascites, prominent collateral vessels, and architectural distortion, all of which suggestive of decompensated cirrhosis. Liver biopsy was not performed due to severe coagulopathy and thrombocytopenia. Despite comprehensive supportive care that included liver-protective agents, diuretics, albumin, and plasma transfusion, her hepatic function progressively declined, and she succumbed to liver failure approximately 180 days after presentation. Postmortem needle necropsy ultimately revealed HAS.
HAS should be considered in the differential diagnosis of rapidly progressive hepatic failure of unknown etiology, especially when imaging results show diffuse hepatic involvement without a clear mass.
肝血管肉瘤（HAS）是一种罕见的、侵袭性很强的血管恶性肿瘤。HAS的临床表现和影像学表现均不具有特异性，常常导致诊断延迟或漏诊。我们报告一例78岁女性的HAS病例，其主要症状为进行性腹胀和厌食。她无饮酒史及肝毒性药物服用史。血清学检测排除了病毒性和自身免疫性肝炎。增强CT未显示明确的肝脏肿块，但发现弥漫性不均匀低密度区，动脉期有斑片状强化，大量腹水，明显的侧支血管以及肝结构扭曲，所有这些提示失代偿期肝硬化。由于严重的凝血功能障碍和血小板减少，未进行肝脏活检。尽管给予了包括肝保护剂、利尿剂、白蛋白和输血在内的全面支持治疗，但其肝功能仍逐渐恶化，在就诊后约180天死于肝衰竭。尸检针吸活检最终确诊为HAS。
在病因不明的快速进展性肝衰竭的鉴别诊断中应考虑HAS，尤其是当影像学结果显示肝脏弥漫性受累且无明确肿块时。
