Durani Urshila, Gallo de Moraes Alice, Beachey Joel, Nelson Darlene, Robinson Steven, Anavekar Nandan S
Department of Internal Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.
Department of Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.
Respir Med Case Rep. 2018 Apr 20;24:77-80. doi: 10.1016/j.rmcr.2018.04.008. eCollection 2018.
Angiosarcomas are rare cancers accounting for less than 2% of all soft tissue sarcomas. We report the case of an unusual presentation of pleural epithelioid angiosarcoma in a patient with constrictive pericarditis and recurrent pleural effusion. A 62 year old smoker presented with acute chest pain. ECG showed diffuse elevation of ST segments in the precordial leads. After extensive evaluation, he was diagnosed with viral pericarditis and treated with colchicine. Two weeks later the patient presented to the emergency department with a large right pleural effusion. Evaluation of the pleural fluid obtained from a thoracentesis revealed an exudative effusion with negative microbial studies and no evidence of malignant cells. His pleural effusion re-accumulated rapidly, requiring repeated thoracenteses over several weeks. Medical thoracoscopy was performed and pleural biopsy revealed primary pleural epithelioid angiosarcoma. Staging PET scan revealed malignant enhancement of right pleura, pericardium, right iliac bone and right shoulder. He died suddenly within 6 weeks of diagnosis, prior to initiating palliative chemotherapy. Pleural angiosarcoma should be considered in the differential diagnosis of recurrent pleural effusions of unknown etiology. Negative cytology does not rule out the diagnosis; excisional biopsy is required. Reported risk factors include asbestos exposure, prior chest radiation, active smoking and history of complicated pleural tuberculosis. Pleural epithelioid angiosarcomas carry a very poor prognosis, with the majority of patients dying within months of diagnosis.
血管肉瘤是罕见的癌症，占所有软组织肉瘤的比例不到2%。我们报告了一例患有缩窄性心包炎和复发性胸腔积液的患者出现不寻常的胸膜上皮样血管肉瘤的病例。一名62岁的吸烟者出现急性胸痛。心电图显示胸前导联ST段广泛抬高。经过广泛评估，他被诊断为病毒性心包炎，并接受秋水仙碱治疗。两周后，患者因大量右侧胸腔积液就诊于急诊科。对胸腔穿刺获取的胸水进行评估，结果显示为渗出性积液，微生物学检查阴性，未发现恶性细胞证据。他的胸腔积液迅速再次积聚，在数周内需要反复进行胸腔穿刺。进行了内科胸腔镜检查，胸膜活检显示为原发性胸膜上皮样血管肉瘤。分期PET扫描显示右侧胸膜、心包、右髂骨和右肩部有恶性强化。他在诊断后6周内突然死亡，尚未开始姑息化疗。在病因不明的复发性胸腔积液的鉴别诊断中应考虑胸膜血管肉瘤。细胞学检查阴性不能排除诊断；需要进行切除活检。报告的危险因素包括接触石棉、既往胸部放疗、主动吸烟和复杂性胸膜结核病史。胸膜上皮样血管肉瘤预后极差，大多数患者在诊断后数月内死亡。
