以顽固性胸腔出血为表现的肺上皮样血管肉瘤1例
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[摘要] 肺上皮样血管肉瘤（PEA）非常罕见，仅见个案报道。目前病因不明确，尚无统一的治疗标准，化疗及放疗都不敏感，临床表现多样，诊断困难。本文介绍1例以顽固性胸腔出血为表现的PEA病例的临床表现、病理、治疗经过及病情进展，进一步加深对该疾病的认识。PEA病理提示广泛出血，发病时有咯血及多发转移者预后极差。
[关键词] 上皮样血管肉瘤；上皮样血管内皮细胞瘤；肺；胸腔出血
[中图分类号] R735.7 [文献标识码] A [文章编号] 1674-4721（2016）02（c）-0148-03
[Abstract] Pulmonary epithelioid angiosarcoma is a kind of malignant tumor which is derived from blood vessel and very rare in clinic ，only case reports.The etiology is not clear，it has no unified standard treatment and is not sensitive to chemotherapy and radiotherapy.Because of the variety of clinical symptoms，it is difficult to diagnosis.1 case of pulmonary epithelioid angiosarcoma with refractory pleural hemorrhage was reported in this paper，in order to provide more clinical information and improve the understanding of the disease.Once pathological prompt extensive hemorrhage，hemoptysis，multiple transfer and the prognosis would be very poor.
[Key words] Epithelioid angiosarcoma；Epithelioid hemangioendothelioma；Lung；Pleural hemorrhage
肺上皮样血管肉瘤（pulmonary epithelioid angiosarcoma，PEA）是一种极为少见的血管源性恶性肿瘤，国内外罕见报道[1-5]。目前病因不明确，治疗困难，预后差，尤其对于病理提示广泛出血，发病即有咯血及多发转移的患者预后更差[5]。诊断主要依据病理，vimentin和CD31敏感性高。由于PEA大多是转移而来，且原发和转移病灶病理学形态类似[6-7]，故在诊断原发性PEA时需首先排除是否存在可疑原发病灶。常见临床表现有持续性咳嗽、血痰、胸痛等，但也可无明显临床症状[8]，因其临床表现多样且无特异性，故容易导致误诊及漏诊，...
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