目的：报道1例肾上腺旁吻合状血管瘤（anastomosing hemangioma, AH）患者，并复习相关文献，对AH的临床病理特征、诊断及鉴别诊断要点进行总结。方法：收集本例AH患者的临床资料，观察其组织学形态及免疫组化特点，并结合相关文献进行综合分析。结果：大体检查见肿瘤位于肾上腺旁脂肪组织内，边界清楚，切面呈灰白、灰红色，质地中等。显微镜下可见肿瘤由相互吻合的窦隙样管腔构成，管腔内衬靴钉样内皮细胞，少数上皮伴有轻度不典型性，可见纤维蛋白性血栓，个别细胞质内可见嗜酸性小体。免疫组化检测示，肿瘤细胞表达CD31和CD34，不表达D2-40、CK5/6、calretinin、CKpan、EMA、Melan-A和HMB45，Ki-67增殖指数约为5%。最终该患者经病理诊断为（肾上腺旁）AH。结果：本文报道1例AH患者，其为罕见的良性血管源性肿瘤，因其血管高度吻合、管腔衬覆的靴钉样内皮细胞常伴不典型的特点，需要与高分化血管肉瘤相区别；泌尿生殖系统的AH还需与血管平滑肌脂肪瘤、腺瘤样瘤、继发于肾细胞癌的血管瘤样增生等疾病相鉴别。

Objective: This paper reported 1 case of paradrenal anastomosing hemangioma (AH), and to summarize the clinicopathological features, diagnosis and differential diagnosis of AH. Methods: The clinical data of this patient was collected, the histological morphology and immunohistochemical characteristics were observed and analyzed in combination with relevant literature. Results: The tumor was located in the adipose tissue around the adrenal gland, it was well circumscribed, gray and red colored. Microscopically, the tumor was composed of irregularly anastomosing sinusoidal-like spaces lined by hobnail endothelial cells, focal mild atypia was observed. Intravascular fibrin thrombi and hyaline globules could be seen. Immunohistochemical results showed diffuse staining for endothelial markers, including CD31 and CD34. D2-40, CK5/6, calretinin, CKpan, EMA, Melan-A and HMB45 immunostains were negative. Ki-67 staining indicated a low proliferative activity. Eventually this patient was diagnosed with AH. Conclusions: AH is a rare but distinct type of vascular neoplasm. Owing to its striking anastomosing appearance, it needs to be distinguished from well differentiated angiosarcoma. In addition, the differential diagnosis of urogenital AH also includes angiomyolipoma, adenomatoid tumor, and hemangiomatoid hyperplasia secondary to renal cell carcinoma.