Epithelioid angiosarcoma of the bone - A series of 10 cases
Epithelioid angiosarcoma of the bone - A series of 10 cases
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DOI：
10.1097/00000478-200306000-00001
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发表时间：
2003-06-01
影响因子：
5.6
通讯作者：
Nielsen, GP
中科院分区：
文献类型：
--
作者：
Deshpande, V;Rosenberg, AE;Nielsen, GP
分析10例骨上皮样血管肉瘤的临床及病理特点。其中男 8 例，女 2 例，年龄 26 岁至 83 岁（平均 62 岁）。四个肿瘤是单发的，六个是多灶性的。在两个会诊病例中，提交的诊断结果是转移癌。显微镜下，肿瘤细胞排列成实性和浸润性片状，大多数情况下存在血管通道或囊性扩张的空间。肿瘤细胞具有丰富的嗜酸性细胞质和大的细胞核，具有开放的染色质和明显的嗜酸性核仁。瘤内出血、中性粒细胞浸润和胞质内腔经常出现。所有 10 个肿瘤的一种或多种内皮标记物均呈阳性，其中 CD31 是最敏感的标记物。七例细胞角蛋白染色呈阳性。三个肿瘤的超微结构检查证实了它们的内皮分化。在没有明显血管分化的情况下，丰富的瘤内出血和瘤内中性粒细胞是有用的辅助形态学特征，可能提示血管起源。六名患者因疾病死亡，一名患者因转移而存活，两名患者目前已无疾病。骨上皮样血管肉瘤应纳入骨上皮样肿瘤的鉴别诊断，内皮标记物应成为其免疫组织化学分析的一部分，以避免由于这些实体的治疗和临床结果存在显着差异而误诊为转移癌。
The clinical and pathologic features of 10 epithelioid angiosarcomas of bone were analyzed. There were eight males and two females who ranged in age from 26 to 83 years (mean 62 years). Four tumors were solitary and six were multifocal. In two consultation cases, the submitted diagnosis was metastatic carcinoma. Microscopically, the tumor cells were arranged in solid and infiltrative sheets, and in most cases vascular channels or cystically dilated spaces were present. The neoplastic cells had abundant eosinophilic cytoplasm and large nuclei with open chromatin and prominent eosinophilic nucleoli. Intratumoral hemorrhage, neutrophilic infiltrates, and intracytoplasmic lumina were frequently present. All 10 tumors stained positive for one or more endothelial markers, with CD31 being the most sensitive marker. Seven cases stained positive for cytokeratin. Ultrastructural examination in three tumors confirmed their endothelial differentiation. In the absence of obvious vascular differentiation, abundant intratumoral hemorrhage and intratumoral neutrophils are useful ancillary morphologic features that may suggest a vascular origin. Six patients are dead of disease, one is alive with metastasis, and two patients are currently disease free. Epithelioid angiosarcoma of bone should be included in the differential diagnosis of epithelioid neoplasms of bone, and endothelial markers should be a part of their immunohistochemical analysis to avoid the misdiagnosis of a metastatic carcinoma because of the significant differences in the treatment and clinical outcomes of these entities.