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目的探讨胸膜上皮样血管内皮细胞瘤(PEH)的临床表现、影像、病理学特征、诊断、鉴别诊断、治疗及预后,提高对该病的诊治水平。方法以“胸膜上皮样血管内皮细胞瘤”为检索词检索了四川大学华西医院2007年1月至2014年12月的住院患者资料,仅有1例呼吸内科收治的患者符合条件,就此回顾性分析其临床特点,并进行相关文献复习。以“胸膜上皮样血管内皮细胞瘤”为检索词检索万方数据库和中国期刊网全文数据库,以“pleural epithelioid hemangioendothelioma”为检索词检索PubM ed、Embase、Ovid及Cochrane数据库,检索时间为1975年1月至2015年4月。结果68岁女性患者,临床表现胸背痛,查体发现除左侧胸腔积液体征外,有新近发生的脊柱侧弯畸形及杵状指。胸部增强CT示左侧胸膜弥漫增厚伴胸腔大量积液,部分包裹,左肺舌叶及下叶压迫性不张,不张舌叶内见密度减低影,右肺散在结节。经胸膜活检获得病理,组织学上查见异型细胞巢,进一步免疫组化染色血小板内皮细胞黏附分子(CD31)蛋白阳性,至此明确诊断。本例患者拒绝化疗,明确诊断11个月后死亡。文献复习显示,在上述数据库中共检索到74篇文献,经筛选后,有26篇文献40例患者明确诊断为PEH且具备相应临床资料,符合检索条件。此类患者主要的临床表现为胸痛及呼吸困难,影像学主要表现为胸腔积液,胸膜不规则增厚,组织病理学呈现血管内皮源性免疫标志物阳性。结论 PEH为罕见的低度恶性血管源性肿瘤,病因不清,临床表现及影像学不具有特征性,与肿瘤胸膜转移、胸膜间皮瘤等疾病易混淆,其诊断主要依靠活检组织病理学检查。现有的治疗手段有限,尚无有效的治疗措施。预后与性别、年龄及病变累计范围等多种因素有关。
Objective To investigate the clinical manifestations, imaging, pathological features, diagnosis, differential diagnosis, treatment and prognosis of pleural epithelioid hemangioendothelioma (PEH) and to improve the diagnosis and treatment of this disease. Methods The data of inpatients from January 2007 to December 2014 in West China Hospital of Sichuan University were searched using the term “pleural epithelioid neoplasm” as the search term, and only 1 patient eligible for respiratory medicine was eligible. In this regard, Sexual analysis of its clinical features, and review of relevant literature. We searched PubMed, Embase, Ovid and Cochrane databases using “pleural epithelioid hemangioendothelioma” as the search term and searched the full-text database of Wanfang Database and China Journal Net with the term “pleural epithelioid hemangioendothelioma” From January 1975 to April 2015. Results 68-year-old female patients, chest and back pain clinical manifestations, physical examination found that in addition to the left pleural effusion signs, the recent occurrence of scoliosis deformity and clubbing. Thoracic enhanced CT showed pleural thickening of the left pleural thickening with a large pleural effusion, parcels, left lung lobes and lower lobes pressure atelectasis, not see the tongue to see the shadow of reduced density, scattered in the right lung nodules. Pathologically confirmed by pleural biopsy, histological see abnormal cell nest, further immunohistochemical staining of platelet endothelial cell adhesion molecule (CD31) protein positive diagnosis so far. In this case, the patient refused chemotherapy and the patient died after a definite diagnosis of 11 months. Literature review showed that in the above database, a total of 74 articles were retrieved. After screening, 40 cases of 26 patients with definite diagnosis of PEH and corresponding clinical data were eligible for retrieval. The main clinical manifestations of these patients were chest pain and dyspnea. Imaging mainly manifested pleural effusion, pleural irregular thickening, and histopathology showed positive endothelium-derived immuno-markers. Conclusions PEH is a rare low-grade malignant tumor of vasculature. Its etiology is unclear, and its clinical manifestations and imaging are not characteristic. It is confused with diseases such as pleural metastasis of tumor and mesothelioma of the pleural space. The diagnosis depends on biopsy histopathology . The existing treatment is limited, there is no effective treatment. Prognosis and gender, age and the cumulative range of lesions and other factors.