Primary Pleural Epithelioid Angiosarcoma Presenting with Persistent Pleural Effusion and Bone Metastases

以持續性肋膜積液及骨頭轉移為表現的原發性肋膜上皮樣血管肉瘤

10.6323/JoCRP.2014.30.1.4

馬維力(Wei-Li Ma)；陳若白(Jo-Pai Chen)

血管肉瘤 ； 內皮細胞標記 ； 化學治療 ； 抗血管新生藥物 ； angiosarcoma ； endothelial marker ； chemotherapy ； anti-angiogenesis treatment

台灣癌症醫學雜誌

30卷1期（2014 / 03 / 01）

35 - 40

英文

我們報告一位中年女性因為持續性的肋膜積液以及骨骼轉移，之後被診斷為原發性肋膜上皮樣血管肉瘤。肋膜積液中不正常細胞表面的內皮細胞標記幫我們確定診斷。然而，儘管這病人接受積極化學治療、抗血管新生藥物、以及肋膜沾黏手術，最後仍因疾病惡化而過世。總結：藉由免疫組織化學染色染出內皮細胞標記對診斷血管肉瘤是重要的；原發性肋膜上皮樣血管肉瘤的預後在血管肉瘤中特別差。對於這種預後差的腫瘤，我們需要更有效的治療。

We report the case of a woman with persistent bilateral pleural effusion and multiple bone metastases. Primary pleural epithelioid angiosarcoma was diagnosed on the basis of endothelial markers expressed on atypical cells from the pleural effusion. Despite intensive chemotherapy, anti-angiogenesis treatment, and surgical pleurodesis, the disease progressed and the patient died. The immunohistochemical stain of endothelial markers is valuable in confirming a diagnosis of angiosarcoma. The prognosis of primary pleural epithelioid angiosarcoma is particularly poor. It is hoped that there will be more effective treatment for this malignancy in the near feature.