原发性肝血管肉瘤2例并文献复习

Primary Hepatic Angiosarcoma: Report of Two Cases and Literature Review

摘要: 目的：探讨原发性肝血管肉瘤(primary hepatic angiosarcoma, PHA)的临床表现及实验室、影像学、病理学检查特点，并复习文献，归纳肝血管肉瘤的诊治新进展。方法：回顾分析两例PHA患者的临床资料，包括临床症状、影像学检查、实验室检查、病理学结果及治疗方案，总结诊治经验。结果：2例患者起病隐匿，早期均无特异性症状；实验室检查方面缺少特异性血清学指标；影像学方面，2例患者一例为单发巨块型肿瘤，一例为巨块结节混合型肿瘤，平扫CT均为不均匀低密度混杂灶，MRI为T1低、稍高信号混杂，T2不均匀高信号灶；增强扫描动脉期肿瘤中心呈条片状弱强化或无强化，周边呈斑点状或结节状强化，瘤内可见分隔，静脉期及延迟期填充式强化并见无强化区；病理学检查是诊断的“金标准”，免疫组化方面可以考虑将ERG蛋白加入检测队列中增加诊断正确率；目前最有效的治疗方式是根治性切除，靶免联合治疗是一种新的治疗选择，可能使中晚期患者获益。结论：PHA是一种罕见高恶性程度的肿瘤，依靠症状及实验室检查诊断困难，确诊需依赖病理学检查，手术切除是治疗首选，靶免联合治疗可能有较好的研究前景。

Abstract: Objective: To explore clinical manifestations and laboratory, imaging, and pathological features of primary hepatic angiosarcoma (PHA), and review the literature to summarize the new advances in diagnosing and treating hepatic angiosarcoma. Methods: We retrospectively analyzed the clinical data of two patients with PHA, including clinical symptoms, imaging examinations, pathological findings, and reviewed the literature for further analysis of this disease. Results: Both two patients had insidious onset and no specific symptoms in the early stage. On imaging, one case was a single giant tumor and the other was a mixed giant nodular tumor, both of which were heterogeneous hy-podense foci on plain CT. MR showed mixed low and slightly high signals in the T1 phase and heter-ogeneous high signal foci in the T2 phase. In the arterial phase, the center of the tumor shows weak or no enhancement, and the periphery shows speckle or nodular enhancement with intra- tumor separation. Filled enhancement and non-enhanced areas can be seen in the venous and delayed phases. The pathological examination is the “gold standard” for diagnosis, and immunohistochem-istry can be considered to add ERG protein to the test cohort to increase the correct diagnosis rate. Currently, the most effective treatment is radical resection, and the combination of targeted thera-py and immunotherapy is a new treatment option that may benefit patients. Conclusions: PHA is a rare and highly malignant tumor, which is difficult to diagnose by symptoms and laboratory exam-ination. Enhanced CT and MRI can better reflect the lesion’s morphology, internal structure, and growth characteristics, but the diagnosis needs to be confirmed by combining them with pathologi-cal examination. Surgical resection is the first choice of treatment, and the combination of targeted therapy with immunotherapy may have better research prospects.