Ha Fu-Shuang, Liu Hua, Han Tao, Song De-Zhao
The Third Central Clinical College of Tianjin Medical University, Tianjin 300170, China.
World J Gastrointest Oncol. 2022 May 15;14(5):1050-1056. doi: 10.4251/wjgo.v14.i5.1050.
Primary hepatic angiosarcoma (PHA) is a rare malignancy with a poor prognosis. It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers, and it rapidly progresses and has a high mortality. To our knowledge, PHA has not been reported to mimic hepatic sinusoidal obstruction syndrome. Herein, we present a case of PHA manifesting as hepatic sinusoidal obstruction syndrome, diagnosed using transjugular liver biopsy, that resulted in the death of the patient.
A 71-year-old man was admitted with the primary complaint of abdominal distension, decreased appetite, fatigue in the previous month, and loss of 10 kg of weight in the past 2 years. Both the liver and spleen were enlarged, and the liver had a medium-hard texture on percussion. Laboratory examinations were performed, and abdominal plain computed tomography (CT) and contrast-enhanced CT showed hepatomegaly and splenomegaly, as well as diffuse low-density shadows distributed in the liver and spleen. Contrast-enhanced CT revealed diffuse, hypodense, nodular or flake shadows in the liver and heterogeneous enhancement in the spleen. A transjugular liver biopsy was performed. Based on the pathology results, the patient was diagnosed with hepatic sinusoidal obstruction syndrome secondary to PHA. The patient's status further deteriorated and he developed serious hepatic failure. The patient was discharged, and died 3 d later.
PHA is rare and has a poor prognosis; however, transjugular liver biopsy can be safely performed to aid in diagnosis.
原发性肝血管肉瘤（PHA）是一种罕见的恶性肿瘤，预后较差。由于缺乏特异性症状或肿瘤标志物，PHA难以诊断，且其进展迅速，死亡率高。据我们所知，尚未有PHA表现为肝窦阻塞综合征的报道。在此，我们报告一例表现为肝窦阻塞综合征的PHA病例，该病例经经颈静脉肝活检确诊，最终患者死亡。
一名71岁男性因腹胀、食欲减退、近1个月疲劳以及过去2年体重减轻10 kg为主诉入院。肝脏和脾脏均肿大，肝脏叩诊质地中等硬度。进行了实验室检查，腹部平扫计算机断层扫描（CT）及增强CT显示肝脏和脾脏肿大，肝脏和脾脏内可见弥漫性低密度影。增强CT显示肝脏内弥漫性低密度、结节状或片状阴影，脾脏强化不均匀。进行了经颈静脉肝活检。根据病理结果，患者被诊断为继发于PHA的肝窦阻塞综合征。患者病情进一步恶化，出现严重肝功能衰竭。患者出院，3天后死亡。
PHA罕见且预后较差；然而，经颈静脉肝活检可安全进行以辅助诊断。
