Surgical Pathology Division, Department of Laboratory, A. Manzoni Hospital, Via dell'Eremo 9/11, 23900 Lecco, Italy.
Pathol Res Pract. 2010 Jun 15;206(6):415-9. doi: 10.1016/j.prp.2009.11.008. Epub 2010 Jan 20.
Malignant vascular tumors are uncommon sarcomas that arise from endothelial cells of small blood vessels and may affect every organ. Pleural localization is very exceptional, and only 48 cases have been reported in the English literature to date. Even if etiological factors implicated in the development of vascular sarcomas are still unclear, the strongest association with the disease was a history of chronic tuberculous pyothorax, observed only in Japanese patients, while prior radiotherapy and occupational exposure to asbestos have been reported in few Western cases. The mean age at diagnosis was 58 years, and the male to female ratio was 6:1. The overall prognosis was poor, and most of the patients died of disease soon after diagnosis. Histological features and clinical presentation often cause several problems in the differential diagnosis, particularly with mesothelioma and metastasis from poorly differentiated carcinomas. Immunohistochemistry plays an important role in identifying these rare entities, confirming the endothelial origin of the neoplasm with the expression of at least one of the vascular markers CD31, CD34, or factor VIII-related antigen. We report herein a further case of a 62-year-old man who presented with progressive dyspnea and bilateral massive hemothorax. The overall pathological and immunohistochemical features of the pleural specimens supported the diagnosis of epithelioid angiosarcoma.
恶性血管肿瘤是一种罕见的肉瘤，起源于小血管的内皮细胞，可能影响到每个器官。胸膜定位非常罕见，迄今为止，英文文献中仅报道了 48 例。尽管血管肉瘤发生的病因因素仍不清楚，但与该病最强的关联是慢性结核性脓胸病史，仅在日本患者中观察到，而在少数西方病例中报道了放射治疗和职业性接触石棉。诊断时的平均年龄为 58 岁，男女比例为 6:1。总体预后较差，大多数患者在诊断后不久因疾病去世。组织学特征和临床表现常导致鉴别诊断中的几个问题，特别是与间皮瘤和低分化癌转移的鉴别。免疫组织化学在识别这些罕见实体中起着重要作用，通过表达至少一种血管标志物 CD31、CD34 或因子 VIII 相关抗原来确认肿瘤的内皮起源。我们在此报告了另一个 62 岁男性患者的病例，该患者表现为进行性呼吸困难和双侧大量血胸。胸膜标本的整体病理和免疫组织化学特征支持上皮样血管肉瘤的诊断。
