Balicki D, Buhrmann R, Maclean J, Cooper B, Minassian H, Wang N S, Hüttner I
Department of Medicine, McGill University, Montreal, Quebec, Canada.
Blood Cells Mol Dis. 1996;22(3):205-13. doi: 10.1006/bcmd.1996.0101.
Epithelioid angiosarcoma of the bone represents a challenging diagnosis by bone marrow biopsy. We present a case of a multicentric high grade angiosarcoma of the bone with epithelioid features. On the basis of the clinical presentation, the radiological findings, and the appearance of loosely clustered tumor cells detected in the initial bone marrow biopsy, the main differential diagnoses considered were a poorly differentiated non-secretory multiple myeloma and metastatic carcinoma. Subsequent morphologic, immunohistochemical and electron microscopic examination of tissue samples clarified the nature of the tumor as epithelioid angiosarcoma. We discuss potential pitfalls in clinical and morphological diagnosis. The strong reactivity of the tumor cells with the nonspecific but ubiquitous mesenchymal marker vimentin in similar cases should direct early attention to the rare malignant bone tumor, epithelioid angiosarcoma, with subsequent confirmation of this diagnosis with specific immunohistochemical endothelial cell markers and/or electron microscopy.
骨上皮样血管肉瘤通过骨髓活检进行诊断具有挑战性。我们报告一例具有上皮样特征的多中心高级别骨血管肉瘤病例。根据临床表现、影像学检查结果以及初次骨髓活检中发现的松散聚集的肿瘤细胞形态，主要鉴别诊断考虑为低分化非分泌性多发性骨髓瘤和转移性癌。随后对组织样本进行的形态学、免疫组织化学和电子显微镜检查明确了肿瘤的性质为上皮样血管肉瘤。我们讨论了临床和形态学诊断中可能存在的陷阱。在类似病例中，肿瘤细胞与非特异性但广泛存在的间充质标志物波形蛋白呈强反应性，这应促使早期关注罕见的恶性骨肿瘤——上皮样血管肉瘤，随后通过特异性免疫组织化学内皮细胞标志物和/或电子显微镜确诊。
