López L, Iriberri M, Cancelo L, Gómez A, Uresandi F, Atxotegui V
Unidad de Patología Respiratoria, Hospital de Cruces, Baracaldo, Vizcaya, Spain.
Arch Bronconeumol. 2004 Apr;40(4):188-90. doi: 10.1016/s1579-2129(06)60213-9.
Pulmonary angiosarcoma is an unusual cause of diffuse pulmonary hemorrhage. Angiosarcomas are rare malignant vascular tumors accounting for 1% to 2% of all sarcomas. Angiosarcomas have been detected in nearly all organs, but lung involvement is unusual, accounting for less than 7%. The literature describes approximately 10 isolated cases of primary pulmonary angiosarcoma as opposed to the more common metastatic type. Given that primary and metastatic types are clinicopathologically similar, the presence of a distant primary sarcoma must be ruled out before a diagnosis of primary pulmonary angiosarcoma can be made. A pathological diagnosis requires a finding of polygonal or oval cells with atypical irregular nuclei and vascular spaces lined with such cells surrounded by hemorrhagic phenomena. Immunohistochemical analysis is positive for specific endothelial cell markers such as CD31 and factor VIII, and coexpression of keratin is a frequent finding.
肺血管肉瘤是弥漫性肺出血的一种罕见病因。血管肉瘤是罕见的恶性血管肿瘤，占所有肉瘤的1%至2%。几乎在所有器官中都检测到过血管肉瘤，但肺部受累情况并不常见，占比不到7%。文献报道了约10例孤立性原发性肺血管肉瘤病例，与之相对的是更常见的转移性类型。鉴于原发性和转移性类型在临床病理上相似，在诊断原发性肺血管肉瘤之前必须排除远处原发性肉瘤的存在。病理诊断需要发现具有非典型不规则核的多边形或椭圆形细胞以及由这些细胞内衬的血管腔，并伴有出血现象。免疫组化分析对特定的内皮细胞标志物如CD31和因子VIII呈阳性，且角蛋白共表达是常见表现。
