Goldblum J R, Rice T W
Department of Pathology, Cleveland Clinic Foundation 44195, USA.
Hum Pathol. 1995 Nov;26(11):1275-7. doi: 10.1016/0046-8177(95)90205-8.
Pulmonary artery angiosarcoma is a rare entity. We report a case of an epithelioid angiosarcoma developing from the right pulmonary artery with pulmonary parenchymal invasion. The patient was a 69-year-old man who presented with massive hemoptysis and shortness of breath. Right middle and lower lobectomies were performed because of uncontrollable bleeding. An angiosarcoma was observed developing from the right pulmonary artery with contiguous spread down smaller artery branches with invasion of the pulmonary parenchyma. Although typical angiosarcomatous areas were observed, the neoplasm was dominated by cells with an epithelioid morphology. Immunohistochemically, the majority of cells stained for endothelial markers factor VIII-related antigen and CD34, but in addition, the cells with an epithelioid morphology stained intensely for cytokeratin. Knowledge of cytokeratin positivity in epithelioid vascular neoplasms is critical to avoid a misdiagnosis of carcinoma, particularly at sites where carcinoma is a much more likely diagnosis.
肺动脉血管肉瘤是一种罕见的疾病。我们报告一例起源于右肺动脉并侵犯肺实质的上皮样血管肉瘤病例。患者为一名69岁男性，表现为大量咯血和呼吸急促。因出血无法控制，进行了右中肺叶和下肺叶切除术。观察到血管肉瘤起源于右肺动脉，沿较小动脉分支连续蔓延并侵犯肺实质。尽管观察到典型的血管肉瘤区域，但肿瘤主要由具有上皮样形态的细胞组成。免疫组化显示，大多数细胞内皮标志物VIII因子相关抗原和CD34染色阳性，但此外，具有上皮样形态的细胞细胞角蛋白染色强烈。了解上皮样血管肿瘤中细胞角蛋白阳性对于避免误诊为癌至关重要，尤其是在癌更可能被诊断的部位。
