Central nervous system metastases of a pulmonary epitheloid haemangioendothelioma
Central nervous system metastases of a pulmonary epitheloid haemangioendothelioma
复制标题
肺上皮样血管内皮瘤的中枢神经系统转移
DOI：
10.1183/09031936.04.00060104
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发表时间：
2004
影响因子：
24.3
通讯作者：
L. Pellín
中科院分区：
文献类型：
--
作者：
R. Díaz;A. Segura;V. Calderero;I. Cervera;J. Aparicio;María V. Jordá;L. Pellín
报告了一例55岁男性右侧胸腔积液和多发性双侧结节的病例。诊断性胸廓切开术是必要的，以获得明确的组织学诊断。在术后过程中，受试者的神经系统状况恶化，并发现多处脑肿块病变。肺和脑肿瘤的病理分析显示非典型的内皮细胞增殖;血管免疫组化标记物，如因子VIII和CD 34，呈强阳性。他的一般状况仍然很差，病人在初步诊断后18个月死亡。最终诊断为肺上皮样血管内皮瘤伴同步中枢神经系统播散，作者首次认为有相关报道。由于其罕见性，对这些肿瘤的预后和治疗知之甚少。消极的预后因素似乎是存在的症状，胸腔积液或多灶性介绍。治疗应包括手术切除，如果可能的话;化疗似乎没有什么效果。观察等待是一种可接受的选择，特别是在无症状患者中。
The case of a 55‐yr-old male with a right pleural effusion and multiple bilateral nodules is reported. A diagnostic thoracothomy was necessary to obtain a definitive histological diagnosis. During the postoperative course, the subject's neurological condition deteriorated and multiple cerebral mass lesions were discovered. The pathological analysis of both lung and cerebral tumours revealed an atypical endothelial cell proliferation; vascular immunohistochemical markers, such as factor VIII and CD34, were strongly positive. His general condition remained poor and the patient died 18 months after the initial diagnosis. The final diagnosis was pulmonary epitheloid haemangioendothelioma with synchronous central nervous system dissemination, the first time the authors believe that association has been reported. Little is known of the prognosis and treatment of these tumours, due to their rarity. Negative prognostic factors appear to be the presence of symptoms, pleural effusion or multifocal presentations. Treatment should include surgical resection if possible; chemotherapy appears to have little effect. Watchful waiting is an acceptable option, especially in asymptomatic patients.