肝臟惡性血管肉瘤是一種源自血管内皮細胞的間質層腫瘤，好發於年紀大的男性，是罕見且高度 惡性的疾病。在病理確認診斷後，病患常因肝臟腫瘤之迅速進展或轉移至其他器官而死亡。因其少見及 高度惡性，故其疾病進展史、預後因子、及治療準則等，到目前為止仍無法建立。本研究採回溯性設計， 收集單一醫學中心，自2003年10月至2013年10月為止的十年期間，所收治的原發性肝臟惡性腫瘤， 包含肝小細胞癌、膽管癌、肝腺癌、肝臟惡性血管瘤、膽管腺癌等，共3560人。其中經病理分析確診為 肝臟惡性血管瘤者有18人，佔0.5%。這群病患的臨床症狀，包括上腹痛（11人），腹脹(5人），且腫瘤破 裂的患者容易有腹痛及腹脹症狀。若依手術可行性分組，一組因多顆腫瘤瀰漫性分布於左右肝葉，或者 侵犯血管，並合併心肺功能不全因素，而無法接受手術者，歸類為未接受癌根除手術(10人），另一組則 能接受癌根除手術(8人）。比較兩組發現，在腫瘤數目(p=.01)與腫瘤位置(p=.02)有明顯差異。在未接受癌 根除手術這組其一年存活率為0%(0-7個月）平均存活僅3.4個月；在接受癌根除手術其第一及二年存活率 為75%，三年存活率為50%，顯著高於未接受癌根除手術患者(p<.01)。目前針對無法接受肝臟惡性血管 肉瘤根除手術的病患，所使用的替代療法，包括化學療法，肝動脈化療栓塞等皆無較好的療效。故吾人 資料顯示，肝臟惡性血管肉瘤的病患若能接受癌根除手術，其預後會比替代療法較好

Hepatic angiosarcoma originated from vascular endothelial cells, is a rare and highly aggressive tumor and often occurs in elderly man. Most patients died of rapid tumor progression or metastases once it has been diagnosed. However, there is no standard treatment guideline towards hepatic angiosarcoma due to rarity of case and poor prognosis. Herein, we conducted a retrospective case review at a single medical center in central Taiwan to evaluate surgical outcomes of patients with primary hepatic angiosarcoma. During the period between November 2003 and November 2013, 3560 patients were diagnosed with hepatic malignancy and 18 (0.5%) of them were pathologically confirmed with hepatic angiosarcoma. Eleven patients experienced abdominal pain and five patients complained abdominal distention. Those symptoms were common in tumor rupture patients. Patients with primary hepatic angiosarcoma were further divided into two groups, with (n = 8) or without curative operation (n = 10) due to diffuse nodularity of multiple liver lobes or vascular invasion and combined with cardiopulmonary dysfunction. Eight patients received curative operation and two of them received liver transplantation due to liver cirrhosis. The number of tumor (p = .01) and single liver lobe (p = .02) were significantly different between two groups. For those who had surgery, one and two-year overall survival rate was 75% and three-year overall survival rate was 50%. The other 10 patients were not operable and were treated with chemotherapy or transcatheter arterial chemoembolization, one-year overall survival rate was 0% and the mean survival time was 3.4 months. Therefore, patients with hepatic angiosarcoma have a better prognosis if they are able to receive surgical resection.