Department of Pathology, Wan Fang Hospital, Taipei Medical University, Taiwan.
Diagn Pathol. 2011 Dec 30;6:130. doi: 10.1186/1746-1596-6-130.
Primary pleural angiosarcoma is a rare and clinically aggressive tumor. Patients usually present with chest pain, dyspnea, hemoptysis and/or cough. Radiologic studies reveal diffuse pleural thickening and pleural effusion with or without mass lesion. The clinical and radiological features both resemble those of mesothelioma, and its definite diagnosis requires careful histologic examination. However, frequent epithelioid feature and immunoreactivity to cytokeratin in primary pleural angiosarcoma further complicate the pathologic diagnosis. The use of proper immunohistochemical stains is often needed to support endothelial differentiation in the tumor cells and to exclude metastatic carcinoma and mesothelioma. We report the case of a 49-year-old male patient with primary pleural angiosarcoma, who presented with initial hemothorax, followed by a rapid progress to an inoperable status.
原发性胸膜血管肉瘤是一种罕见且具有侵袭性的肿瘤。患者通常表现为胸痛、呼吸困难、咯血和/或咳嗽。影像学研究显示弥漫性胸膜增厚和胸腔积液,伴或不伴肿块。其临床和影像学特征均类似于间皮瘤,明确诊断需要仔细的组织学检查。然而,原发性胸膜血管肉瘤中常见的上皮样特征和细胞角蛋白免疫反应进一步使病理诊断复杂化。通常需要使用适当的免疫组织化学染色来支持肿瘤细胞的内皮分化,并排除转移性癌和间皮瘤。我们报告了一例 49 岁男性原发性胸膜血管肉瘤患者,其最初表现为血胸,随后迅速发展为无法手术的状态。