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.