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目的:提高对以附睾囊肿为首发症状的von Hippel-Lindau(VHL)综合征的认识水平。方法:手术治疗3例附睾囊肿患者,对附睾组织进行常规病理和免疫组学检测,并加强术后随访观察。结果:3例均行手术切除附睾囊肿,附睾组织常规病理检查显示囊壁被覆上皮乳头样增生,进一步行免疫组化检查显示缺氧诱导因子(HIF-1α)、血管内皮生长因子(VEGF)、α平滑肌动蛋白(α-SMA)、CD34等表达均为阳性。随访期内,3例患者分别因VHL综合征家族史并肾癌、视网膜血管网织细胞瘤并肾癌、视网膜血管网织细胞瘤并肾上腺嗜铬细胞瘤而被确诊为VHL综合征。结论:附睾囊肿病理检查异常时应进一步作免疫组化分析,若结果提示HIF-1α、VEGF、α-SMA、CD34阳性表达时,应想到VHL综合征的可能性,并完善相关检查或加强术后随访。
OBJECTIVE: To raise awareness of von Hippel-Lindau (VHL) syndrome, a first symptom of epididymal cysts. Methods: Three patients with epididymal cyst were surgically treated. The epididymal tissues were examined by routine pathology and immunohistochemistry. Postoperative follow-up was also observed. Results: The epididymal cysts were removed in 3 cases. The pathological examination of the epididymis showed epithelial papillary hyperplasia on the wall of the epididymal mucosa. Further immunohistochemistry showed that the expression of hypoxia inducible factor (HIF-1α), vascular endothelial growth factor (VEGF) α-smooth muscle actin (α-SMA), CD34 expression were positive. During the follow-up period, 3 patients were diagnosed with VHL syndrome due to the family history of VHL syndrome with renal cancer, retinoblastoma and renal cell carcinoma, retinoblastoma and adrenal pheochromocytoma, respectively. Conclusion: The pathological examination of epididymal cyst should be further analyzed by immunohistochemistry. If the results suggest that the expression of HIF-1α, VEGF, α-SMA and CD34 are positive, we should think about the possibility of VHL syndrome and improve the related inspection or enhancement Follow-up.