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伴有肺部受累的原发性胸膜上皮样血管内皮瘤

Primary Pleural Epitheliod Hemangioendothelioma with Lung Involvement.

作者信息

Siraj Saima, Akhter Saima, Rizvi Nadeem

机构信息

Department of Chest Medicine, Jinnah Postgraduate Medical Centre, Karachi.

出版信息

J Coll Physicians Surg Pak. 2017 Sep;27(9):S120-S121.

PMID:28969747
Abstract

The authors report a case of 50-year man who presented with 2-year history of dry cough, right sided chest pain, and shortness of breath. Chest X-ray revealed right-sided pleural effusion and left-sided opacity. Pleuroscopic pleural biopsy confirmed the diagnosis of primary pleural epitheliod hemangioendothelioma (EHE) with peripheral lung parenchymal invasion. Chest drain was inserted; and significant amount of fluid was drained, but lung failed to expand after 72 hours. Patient was planned for video assisted thoracoscopy (VATS) and also discussed with oncology department for chemotherapy; but he refused any further treatment, and left home against medical advice with chest drain in place. EHE originating from pleura is extremely rare with an aggressive clinical course and poor prognosis. To our knowledge, this is the first reported case of an EHE originating from pleura in South Asia and highlights the heterogeneous geographic distribution of tumor and demonstrates the need for a more systemic approach to all patients with unilateral pleural effusion.

摘要

作者报告了一例50岁男性,有2年干咳、右侧胸痛和气短病史。胸部X线显示右侧胸腔积液和左侧肺部致密影。胸腔镜胸膜活检确诊为原发性胸膜上皮样血管内皮瘤(EHE)伴外周肺实质侵犯。插入胸腔引流管;引出了大量液体,但72小时后肺仍未复张。计划为患者行电视辅助胸腔镜手术(VATS),并与肿瘤科讨论化疗;但他拒绝进一步治疗,不顾医嘱带着胸腔引流管回家了。起源于胸膜的EHE极为罕见,临床病程侵袭性强,预后差。据我们所知,这是南亚地区首例报告的起源于胸膜的EHE病例,突出了肿瘤的异质性地理分布,并表明需要对所有单侧胸腔积液患者采取更系统的方法。

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