Department of Medicine, Santa Clara Valley Medical Center, San Jose, CA.
Department of Medicine/Oncology, Stanford Medical Center, Stanford, CA.
Am J Clin Oncol. 2023 Oct 1;46(10):439-444. doi: 10.1097/COC.0000000000001032. Epub 2023 Aug 15.
Primary hepatic angiosarcoma is a rare tumor of the liver that originates from endothelial and fibroblastic tissue, with poor prognosis and lack of standardized treatment. We retrospectively analyzed the clinical characteristics and treatment outcomes of 23 patients with primary liver angiosarcoma treated at an academic sarcoma center.
We screened all patients with primary liver angiosarcoma treated at Stanford between 2000 and 2022. Data was collected from EPIC electronic medical records and included patient demographics, tumor characteristics, treatment modalities, and patient outcomes. Statistical analysis was completed using Python 3.0, while survival curves were generated using the Kaplan-Meier method and Lifelines Packages.
There were nearly equal numbers of males (11) and females (12) in our study, with most patients aged 70 to 79 at diagnosis. The median overall survival (OS) was 6 months (range 0.07 to 222.6 mo). The 2- and 5-year OS were both 38.6%. 71% of patients received systemic treatment with chemotherapy, while 29% received immunotherapy. Local treatment with surgery or radioembolization was performed in 14% of patients. Three patients in our study displayed particularly improved OS and received various treatments, which ranged from hepatic resection to ipilimumab/nivolumab.
Our study demonstrated that primary liver angiosarcoma has poor outcomes despite treatment. Surgical resection with negative margins is the only curative modality. However, most patients present with advanced disease and are not surgical candidates. Further research is needed to identify more effective systemic therapy options for this devastating disease.
原发性肝血管肉瘤是一种罕见的肝脏肿瘤，起源于内皮和纤维母组织，预后较差，缺乏标准化治疗。我们回顾性分析了在一家学术肉瘤中心治疗的 23 例原发性肝血管肉瘤患者的临床特征和治疗结果。
我们筛选了斯坦福大学 2000 年至 2022 年间所有治疗的原发性肝血管肉瘤患者。数据来自 EPIC 电子病历，包括患者人口统计学特征、肿瘤特征、治疗方式和患者结局。使用 Python 3.0 完成统计分析，使用 Kaplan-Meier 方法和 Lifelines 包生成生存曲线。
本研究中男性和女性患者数量几乎相等（分别为 11 例和 12 例），大多数患者诊断时年龄为 70 至 79 岁。中位总生存期（OS）为 6 个月（范围 0.07 至 222.6 个月）。2 年和 5 年 OS 均为 38.6%。71%的患者接受了化疗的系统治疗，而 29%的患者接受了免疫治疗。14%的患者接受了手术或放射栓塞的局部治疗。我们研究中的 3 名患者的 OS 特别改善，接受了各种治疗，包括肝切除术、ipilimumab/nivolumab。
尽管进行了治疗，但原发性肝血管肉瘤的预后仍然很差。阴性切缘的手术切除是唯一的治愈方式。然而，大多数患者患有晚期疾病，不适合手术。需要进一步研究以确定针对这种毁灭性疾病更有效的全身治疗选择。
