[Analysis of clinicopathological features of 18 cases of hepatic angiosarcoma].

Objective: To investigate the clinicopathological features, treatment, and prognosis of hepatic angiosarcoma. Methods: Clinicopathological data and prognostic conditions of 18 cases with hepatic angiosarcoma were collected retrospectively. The recurrence-free survival rate and overall survival rate were calculated by the Kaplan-Meier method. A Cox regression analysis was used to explore the survival-related risk factors. Results: There were 12 male and 6 female patients, with an average age of 57 (37 ~ 70) years. The tumor's average diameter was 8.40 (2.00 ~ 18.00) cm. Seven cases had multiple tumors, while two cases had large vessel tumor thrombuses. Microscopically, the tumor tissues were irregularly anastomosed, with vascular lacunar or solid bundle-like weaving, and the tissue morphology mimicked capillary hemangioma, cavernous hemangioma, or angioepithelioma, while tumor cells were spindle-shaped or epithelioid, lined with hobnails in the lumen, or formed papillary structures in the lumen. The proportion of highly, moderately, and poorly differentiated tumors was 4:8:6, with six cases having clear tumor boundaries, eight having microvascular tumor thrombi, and sixteen having blood lake formation. Different levels of expression of CD31, CD34, erythroblast transformation-specific related genes, and Fli-1 markers were demonstrated in all of the cases. Four cases had a P53 mutation, and six cases had Ki-67 > 10%. During the follow-up period of 0.23-114.20 months, the five-year recurrence-free survival rate and overall survival rate were 16.7% and 37.2%, respectively. Cox regression multivariate analysis showed that preoperative symptoms and multiple tumors were significant risk factors for recurrence-free survival, while preoperative symptoms and Ki-67 > 10% were significant risk factors for overall survival. Conclusion: Hepatic angiosarcoma is a rare hepatic mesenchymal tumor with high malignancy and a poor prognosis. Pathological morphology and immunohistochemical marker combinations are needed for a definite diagnosis. However, the complexity of angiosarcomas' histological and cytological conformations and the overlap of pathological features with benign vascular tumors, sarcomas, and carcinomas pose difficulties in the differential diagnosis. Thus, the only effective ways to prolong survival are early detection and radical surgical resection.目的： 探讨肝血管肉瘤的临床病理特征和治疗预后。 方法： 回顾性收集18例肝血管肉瘤患者的临床病理信息和预后情况，Kaplan-Meier法计算无复发生存率和总生存率，Cox回归分析探索生存相关风险因素。 结果： 患者男性12例，女性6例，平均年龄57（37~70）岁。肿瘤平均直径8.40 （2.00~18.00）cm，多发肿瘤7例，2例有大血管瘤栓。镜下肿瘤组织排列呈不规则吻合状血管腔隙样或实性束状编织状，组织形态上可模拟毛细血管瘤、海绵状血管瘤或血管外皮瘤。瘤细胞可呈梭形或上皮样，呈靴钉样衬覆于管腔或在管腔内形成乳头状结构。高、中、低分化肿瘤占比为4∶8∶6，6例肿瘤边界清晰，8例有微血管瘤栓，16例见血湖形成。全部病例不同程度表达CD31、CD34、成红细胞转化特异性相关基因、Fli-1标志物，P53突变病例4例，Ki-67 > 10%病例6例。随访0.23~114.20个月，5年无复发生存率为16.7%，5年总生存率为37.2%。Cox回归多因素分析提示，术前有症状、多发肿瘤为无复发生存的显著风险因素，术前有症状、Ki-67 > 10%为总生存的显著风险因素。 结论： 肝血管肉瘤是一种罕见的肝脏间叶源性肿瘤，恶性程度高，预后差，确诊需结合病理形态和免疫组织化学标志物情况。复杂的组织和细胞学构象以及与良性血管源性肿瘤、肉瘤、癌在病理特征上的重叠是血管肉瘤鉴别诊断的难点。早期发现和根治性手术切除是延长生存期仅有的有效方法。.