Aggressive clinical course of epithelioid angiosarcoma in the femur: a case report.
Aggressive clinical course of epithelioid angiosarcoma in the femur: a case report.
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DOI：
10.1186/1477-7819-12-281
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发表时间：
2014-09-11
影响因子：
3.2
通讯作者：
Iwamoto Y
中科院分区：
文献类型：
--
作者：
Sakamoto A;Takahashi Y;Oda Y;Iwamoto Y
上皮样血管肉瘤是血管肉瘤的一种罕见变体，其特征是模仿癌的上皮样形态外观。这些肿瘤通常发生在骨骼外部位；起源于骨骼的情况很少见。一名 69 岁女性因右膝疼痛就诊。平片和磁共振成像显示溶骨性病变，有大的软组织延伸到股骨远端。根据活检标本诊断为来源不明的转移癌，进行了切除和人工关节置换。切除材料的组织学分析证实为上皮样血管肉瘤，并得到细胞角蛋白和血管标志物免疫表达的支持。手术后三个月，观察到骨和淋巴结转移，不久患者就因病死亡。骨上皮样血管肉瘤的特点是具有侵袭性的临床病程。在具有此类上皮特征的病例中，应考虑骨上皮样血管肉瘤的可能性，特别是在只有小样本的情况下。
Epithelioid angiosarcoma is a rare variant of angiosarcoma, and is characterized by an epithelioid morphologic appearance that mimics carcinoma. These tumors usually arise in extraskeletal sites; origination in bone is rare. A 69-year-old woman presented with right knee pain. Plain radiographs and magnetic resonance imaging showed an osteolytic lesion with a large soft-tissue extension into the distal femur. Under a diagnosis of metastatic carcinoma of unknown origin based on the biopsy specimen, resection and replacement with an artificial joint were performed. Histologic analysis of the resected material confirmed epithelioid angiosarcoma, supported by immunoexpression of cytokeratins and vascular markers. Three months after surgery, metastasis to the bone and lymph nodes was observed, and the patient died of the disease shortly thereafter. Epithelioid angiosarcoma of bone is characterized by an aggressive clinical course. A possibility of epithelioid angiosarcoma of bone should be considered in cases with such epithelial features, particularly if only small specimens are available.
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