骨原发性上皮样血管肉瘤临床病理观察

Primary epithelioid angiosarcoma of bone: a clinicopathologic analysis

目的探讨骨原发性上皮样血管肉瘤的临床特征、影像学表现、病理形态特点、诊断与鉴别诊断及预后。方法分析1例骨原发性上皮样血管肉瘤，结合病理形态特点和免疫组化与其他肿瘤鉴别诊断，并进行文献复习。结果镜下肿瘤细胞上皮样，轻一中度异型，核分裂象易见，呈片状、巢状、条索状排，部分区域可见大小不等、形态不规则分支状血管性腔隙，内衬异型瘤细胞，亦可见单个瘤细胞或多个瘤细胞形成空腔包绕红细胞。免疫组化：肿瘤细胞vimentin、CD31和AE1／AE3均（＋），CK8／18部分（＋），CD34和F8（-）；网状纤维染色血管腔隙结构（＋）。结论原发于骨的上皮样血管肉瘤是一种少见的高度恶性血管源性肿瘤，诊断要结合临床及影像学资料，免疫组化及网状纤维染色有助于明确诊断，并可与转移癌、上皮样血管内皮瘤、恶性问皮瘤、恶性黑色素瘤、间变性大细胞淋巴瘤等鉴别。

Objective To study the clinical, imageologic, pathological characteristics, prognosis and differential diagnosis of primary epithelioid angiosarcoma of bone. Methods A case of epithelioid angiosarcoma of bone was investigated by light microscopy and immunohistochemistry, and the differential diagnosis from other tumors was discused with review of the literatire. Results Histologically, epithelioid angiosarcoma contained large, pleomorphic, round to polygonal epithelioid cells filled with abundant eosinophilic cytoplasm and with increased mitotic figures. The vasoformative structures ranged from well-formed vessel to slit-like anastomosing vascular channels lined by spindle or plump endothelial cells. Cells with intracytoplasmic lumina containing erythrocytes could usually be identified. The tumor cells were positive for vimentin, CD31 and AE1/AE3. Conclusions Epithelioid angiosarcoma of bone is a rare and high aggressive tumor. An accurate diagnosis is based on histopathology, clinical presentations, imaging, immunohistochemical and reticulin staining. The differential diagnosis of epithelioid angiosarcoma includes metastatic carcinoma, epithelioid hemangioendothelioma, mesothelioma, melanoma, anaplastic large cell lymphoma and so on.