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目的:探讨原发性肾脏血管肉瘤的临床、病理学特点及其鉴别诊断和预后评估。方法:在光学显微镜下观察3例肾脏血管肉瘤组织的病理形态,行免疫组织化学(免疫组化)染色检测,并结合文献进行分析讨论。结果:镜下观察,可见肿瘤边界不清,形成不规则的互相吻合的血管腔隙,内衬异型增生的内皮细胞,其中2例伴有上皮样特征。免疫组化检测结果显示,3例肿瘤细胞均为波形蛋白(+)、CD31(+)、CD34(+),例2同时存在少量肿瘤细胞EMA(+),例3同时存在肿瘤细胞AE1/AE3(+)、CK8/18(+),而例1肿瘤细胞上皮性标记AE1/AE3、EMA均为阴性。结论:原发性肾脏血管肉瘤罕见,术前影像学检查缺乏特异性征像,故组织病理及免疫组化检测对患者的最终确诊至关重要。目前较为肯定的治疗方法是外科手术完整切除。
Objective: To investigate the clinical and pathological characteristics of primary renal angiosarcoma and its differential diagnosis and prognosis. Methods: The pathological changes of 3 cases of renal angiosarcoma were observed under optical microscope. Immunohistochemistry (IHC) staining was used to detect the pathological changes. Results: Microscopically, the tumor border was unclear, forming irregular blood vessel cavities that coincided with each other and lined with dysplastic endothelial cells, of which 2 cases were associated with epithelial-like features. Immunohistochemical results showed that all of the three tumor cells were vimentin (+), CD31 (+), CD34 (+), while there were a small number of EMA (+) tumor cells in group 2. Case 3 also had AE1 / AE3 (+), CK8 / 18 (+), while the tumor cell epithelial markers AE1 / AE3 and EMA in Example 1 were negative. CONCLUSIONS: Primary renal angiosarcoma is uncommon and preoperative imaging does not have specific signs. Therefore, histopathology and immunohistochemistry are crucial for the final diagnosis of patients. The current treatment is more sure surgical resection.