Pathologic quiz case: a 39-year-old man with diffuse pleural thickening and massive hemothorax. Epithelioid angiosarcoma of pleura.

Department of Pathology and Laboratory Medicine, Nassau University Medical Center, East Meadow, NY 11554, USA.
Archives of pathology & laboratory medicine (Impact Factor: 2.88). 12/2004; 128(11):1299-300. DOI: 10.1043/1543-2165(2004)128<1299:PQCAYM>2.0.CO;2
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    ABSTRACT: 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.
    Pathology - Research and Practice 06/2010; 206(6):415-9. DOI:10.1016/j.prp.2009.11.008 · 1.56 Impact Factor
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    ABSTRACT: Epithelioid angiosarcoma is a rare malignant vascular tumor with poor prognosis. Pleural or pericardial angiosarcomas are usually secondary tumors. We report a unique case of primary epithelioid angiosarcoma of the pleura and the pericardium in a 28-year-old man who was admitted for Pancoast Tobias syndrome. Thoracoscopy revealed a multifocal neoplasm and pleural biopsy showed a malignant tumor which histological and immunophenotypical features were characteristic of epithelioid angiosarcoma.
    Indian Journal of Thoracic and Cardiovascular Surgery 03/2009; 25(1):34-37. DOI:10.1007/s12055-009-0009-9
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    ABSTRACT: 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.
    Diagnostic Pathology 12/2011; 6(1):130. DOI:10.1186/1746-1596-6-130 · 2.41 Impact Factor