Abstract：
Angiosarcomas involving the liver can be hepatic primary or metastasis from another anatomic site, which have not been systematically compared. We analyzed a series of liver biopsy or resection specimens carrying a diagnosis of angiosarcoma collected between 2005 and 2022 at 3 tertiary medical centers. The cohort included 32 patients (20 M and 12 F) with a median age of 64 years. Nineteen were primary hepatic angiosarcoma (PHA) and 13 metastatic angiosarcoma to liver (MA). Males were predominant in PHA (15/19, 78%) compared to MA (5/13, 38%, P = .025). There was no age difference between the 2 groups. Five cases had background hepatic cirrhosis, which more likely harbored PHA (4/5, 80%). Multifocality and multiorgan involvement were common in both groups. Tumor size was significantly larger in PHA than in MA (10.4 versus 4.7 cm, P < .01). Histologically, there were no differences in terms of tumor morphology (spindled versus epithelioid) and growth patterns (vasoformative versus solid) between the 2 groups. Immunohistochemically, all tumor cells were positive for CD31 (28/28, 100%) and ERG (18/18, 100%). Molecular analysis in 5 cases demonstrated different mutation profiles involving different genes, including MTOR, PIK3CA, ARID1A, CDKN2A, PTEN, TP53, ATRX, KDR/VEGFR2, and so forth. On follow-up, 30 patients (93%) died of disease, with a median survival of 114 days. Univariate and multivariate analyses revealed PHA and epithelioid morphology to be associated with worse survival (P < .05), while treatment was associated with better survival (P < .001). Our results confirmed that angiosarcoma, particularly PHA, is extremely aggressive. Epithelioid morphology is an adverse prognosticator and may be used for tumor subclassification.
摘要：
累及肝脏的血管肉瘤可以是原发性肝或从另一个解剖部位转移，没有系统地比较。我们分析了2005年至2018年期间在3个三级医疗中心收集的一系列携带血管肉瘤诊断的肝活检或切除标本。该队列包括32例患者(20M，12F），中位年龄为64岁。19例为原发性肝血管肉瘤(PHA),13例为肝转移瘤(MA)。男性在PHA（15/19，78%）中占主导地位，而MA（5/13，38%，P=0.025)。两组之间没有年龄差异。5例有背景肝硬化，更有可能携带PHA(4/5，80%)。两组中都有多病灶和多器官受累。PHA的肿瘤大小明显大于MA（10.4vs.4.7cm,P<0.01)。组织学上,在肿瘤形态方面没有差异(纺锤状与上皮样)和生长模式(血管形成性与solid)betweenthetwogroups.免疫组织化学，所有肿瘤细胞CD31(28/28,100%)和ERG(18/18,100%)均为阳性。5例的分子分析显示涉及不同基因的不同突变谱，包括MTOR，PIK3CA,ARID1A,CDKN2A,PTEN,TP53、ATRX、KDR/VEGFR2等.关于后续行动，30例（93%）患者死于疾病，中位生存期为114天。单因素和多因素分析显示，PHA和上皮样形态与较差的生存率相关（P<0.05），而治疗与较好的生存率相关（P<0.001）。我们的结果证实了血管肉瘤，尤其是PHA非常具有侵略性。上皮样形态是一种不良的预后因素，可用于肿瘤亚分类。
