Bocchino M, Barra E, Lassandro F, Ranieri F, Muto R, Rea G
Department of Clinical and Experimental Medicine, "Federico II" University, Naples, Italy.
Monaldi Arch Chest Dis. 2010 Sep;73(3):135-9. doi: 10.4081/monaldi.2010.298.
Primary epithelioid haemangioendothelioma (EHE) of the pleura is a rare vascular tumour that occurs mainly in men. Pleural effusion and thickening are the most common clinical presentations. A 58 year old female, nonsmoking patient presented to us with dry cough, dyspnoea and left chest pain for several weeks (no asbestos exposure). Standard chest X-ray and contrast enhanced multislice computed tomography revealed a large-size lobulated mass originating from the pleura which was diagnosed as primary pleural haemangioendothelioma (PHE) by histology and immunohistochemistry (reactivity for vimentin, CD31, CD34, Factor VIII and ulex europeaus). No metastases were detected. The patient refused treatment and died three months later due to the onset of acute and progressive respiratory failure. Despite the lack of high-grade malignancy, primary PHE displays a poor prognosis while curative therapies are actually not available. To our knowledge, this is the first case of primary PHE in a female patient occurring in Italy and the third one to have been reported in English literature. Difficulties in diagnosis and treatment management are discussed below.
原发性胸膜上皮样血管内皮瘤（EHE）是一种罕见的血管肿瘤，主要发生于男性。胸腔积液和增厚是最常见的临床表现。一名58岁不吸烟女性患者因干咳、呼吸困难和左胸痛数周前来就诊（无石棉接触史）。标准胸部X线和增强多层计算机断层扫描显示一个起源于胸膜的大的分叶状肿块，经组织学和免疫组织化学检查（波形蛋白、CD31、CD34、第八因子和欧洲荆豆凝集素呈阳性反应）诊断为原发性胸膜血管内皮瘤（PHE）。未检测到转移。患者拒绝治疗，3个月后因急性进行性呼吸衰竭死亡。尽管原发性PHE缺乏高级别恶性特征，但其预后较差，目前尚无治愈性疗法。据我们所知，这是意大利首例女性原发性PHE病例，也是英文文献中报道的第三例。下文将讨论诊断和治疗管理方面的困难。
