探讨原发性肝血管肉瘤（PHA）的临床病理特征及手术疗效。

回顾性分析2000年1月至2019年12月在西安交通大学第一附属医院行手术切除并经病理检查确诊的4例PHA患者临床资料。患者均签署知情同意书，符合医学伦理学规定。同时在中国知网、万方数据库、PubMed检索主题词为"原发性肝血管肉瘤"、"primary hepatic angiosarcoma"的中、英文文献，纳入经手术切除并经病理检查确诊的49例病例资料。检测肿瘤内CD31、CD34、Vimentin、凝血因子Ⅷ（FⅧ）、Ki-67等表达，总结分析PHA的临床病理特征及预后。

共53例PHA患者纳入研究，其中男26例，女27例；平均年龄（57±3）岁。腹痛28例，腹胀或上腹部不适5例，发热1例，腹部包块1例，腰痛1例，季肋区疼痛1例，咳嗽伴胸闷气急1例，无明显临床症状15例。单发37例，多发16例。肿瘤位于左半肝12例，右半肝32例，肝中叶1例，双侧肝叶3例，全肝叶2例，3例未提供肿瘤部位。肿瘤最大直径1.7~25.0 cm。肿瘤为实性，质中，有包膜。肿瘤细胞呈卵圆形或短梭形，片状或交错排列，异型明显。免疫组化染色示：CD31阳性率78%（21/27），CD34阳性率78%（21/27），Vimentin阳性率58%（21/36），FⅧ阳性率44%（12/27），Ki-67阳性率30%（8/27）。49例文献报道病例手术切除术后，13例行预防性静脉化疗，6例行预防性介入治疗。4例本院病例未进行化疗及介入治疗。随访期间复发1例，肝衰竭2例，转移1例。

PHA临床少见，缺乏特异性临床表现及检测指标，早期诊断困难，易与其他肝脏肿瘤混淆而误诊。手术切除仍是主要治疗手段，确诊依靠病理学检查，手术切除配合术后规律随访疗效确切，可改善预后。

To investigate the clinicopathological features and surgical efficacy of primary hepatic angiosarcoma (PHA).

Clinical data of 4 patients who were pathologically diagnosed with PHA and underwent surgical resection in the First Affiliated Hospital of Xi'an Jiaotong University from January 2000 to December 2019 were retrospectively analyzed. The informed consents of all patients were obtained and the local ethical committee approval was received. Literature was retrieved in CNKI, Wanfang Data and PubMed using the keywords of "primary hepatic angiosarcoma" in both Chinese and English. 49 patients who were pathologically diagnosed with PHA and underwent surgical resection were included. The expression levels of CD31, CD34, Vimentin, coagulation factor Ⅷ (FⅧ) and Ki-67 in the tumor were quantitatively detected. Clinicopathological features and clinical prognosis of PHA were summarized and analyzed.

A total of 53 patients with PHA were included in the study, 26 male and 27 female, aged (57±3) years on average. Abdominal pain was observed in 28 cases, abdominal distension or upper abdominal discomfort in 5, fever in 1, abdominal mass in 1, lumbago in 1, costal region pain in 1, cough complicated with chest tightness and shortness of breath in 1, and no evident clinical symptoms in 15. 37 cases were diagnosed with single onset and 16 cases of multiple onset. The tumors of 12 cases were located in the left lobe, 32 in the right lobe, 1 in the middle lobe, 3 in the bilateral lobes, 2 in the whole lobe, and tumor location in 3 cases was not described. The maximum diameter of tumor was ranged from 1.7 to 25.0 cm. The tumor was solid, moderate texture and encapsulated. The tumor cells presented in oval or short-spindle shape, arranged in lamellar or staggered pattern, with significant cellular atypia. Immunohistochemical staining showed that the positive rate of CD31 was 78%(21/27), CD34 was 78%(21/27), Vimentin 58%(21/36), FⅧ 44%(12/27) and Ki-67 30%(8/27). Among 49 patients undergoing surgical resection, 13 cases were treated with preventive intravenous chemotherapy and 6 cases received preventive interventional therapy. 4 patients in our hospital were not treated with chemotherapy or interventional therapy. During the follow-up, 1 patient recurred, 2 cases developed liver failure and 1 metastasis.

PHA is rarely seen in clinical practice and is lack of specific clinical manifestations and diagnosis parameters. It is difficult to deliver prompt diagnosis and easily misdiagnosed as other liver tumors. Surgical resection is the main treatment. The diagnostic confirmation relies on the pathological examination. Surgical resection combined with regular postoperative follow-up can yield definite efficacy and improve the clinical prognosis.