肺上皮样血管肉瘤临床病理观察  

Epithelioid angiosarcoma of lung:a clinicopathologic analysis

作　　者：陈国勤[1] 刘桂红[2] 莫明聪[1] 何萍[1] 顾莹莹[2] 姜桔红[1] 刘蓉[2] 

机构地区：[1]广州医学院第一附属医院病理科,广州510120 [2]广州呼吸疾病研究所,广州510120

出　　处：《诊断病理学杂志》2013年第2期81-84,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨肺上皮样血管肉瘤(PEA)的临床病理特征、生物学行为和鉴别诊断。方法分析1例肺上皮样血管肉瘤的临床表现、组织学特征、免疫组化结果并行文献复习。结果本例镜下显示瘤组织大部分以梭形细胞为主,弥漫片状分布,形似纤维肉瘤,可见瘤细胞形成的空泡状单细胞性血管腔,部分呈上皮细胞巢状结构的区域见瘤组织呈条索状分布,背景广泛黏液样变,坏死及核分裂易见,并伴有淋巴结转移。免疫组化示肿瘤细胞vimentin、CD34、和CD31(+),EMA少量灶性(+);Ki-67增殖指数不均一,梭形细胞区20%～70%。结论肺上皮样血管肉瘤罕见,具有独特的病理形态学特征,高度恶性,预后不良。需要与上皮样血管内皮细胞瘤、纤维肉瘤、恶性孤立性纤维性肿瘤、低分化腺癌、恶性间皮瘤、转移性胃肠间质瘤等相鉴别。Objective To explore the clinicopathological characteristics of primary epithelioid angiosarcoma （PEA） of lung. Methods The clinical and histological characteristics and immunohistochemical findings were analyzed in one case of PEA sample through review of the literature. Results The morphology of PEA in the lung was characterized by spindle cell, diffusely distributed with fibrosarcoma-like morphology. The vacuolar monocyte vascular lumen formed by tumor cells were observed while tumor tissue distributed in sheet in some nest-like regions with extensive mucoid background, with necrosis and karyokinesis and lymph node metastasis. The immunohistochemistry indicated that the tumor ceils were positive for vimentin, CD34, and CD31, and partially positive for EMA. Ki67 labelling index was various. 20% -30% in spindle cell region. Conclusion The PEA in lung is rare and highly malignant with specific pathological characteristics. The differential diagnosis includes epithelioid hemangioendothelioma, fibrosarcoma, malignant solitary fibroma, poorly- differentiated adenocarcinoma, malignant mesothelioma, andmetastatic gastrointestinal stromal tumors of the lung.

关 键 词：上皮样血管肉瘤 肺 诊断 鉴别诊断 

分 类 号：R734.2[医药卫生—肿瘤]