目的 探索 claudin-5(CLN5)在成血管母细胞瘤中的表达及其在瘤囊形成过程中的意义。方法 本研究11例临床标本均取自无术前激素治疗及放射治疗的小脑成血管母细胞瘤患者,其中4例患者伴有von Hippel-Lindau病。根据患者术前影像学检查,将肿瘤的大体表现分为三型:S型,以实体肿瘤为主要表现;SC型,有较大的实体肿瘤,同时也伴有明显瘤囊形成;C型,以一个较大瘤囊形成为主要表现,肿瘤本身仅表现为囊壁的瘤结节。肿瘤组织标本采用ABC免疫组化的方法来检测标本微血管内皮细胞上CLN5的表达。根据标本微血管内皮细胞阳性率,将免疫组化结果分为3级:阴性,标本中微血管内皮细胞阳性率≤5%;弱阳性,5%<标本中微血管内皮细胞阳性率<30%;强阳性,标本中微血管内皮细胞阳性率≥30%。结果 采用Sperman相关性检验进行统计分析。结果 4例S型及2例SC型患者标本中均可检测到明显的CLN5表达。4例C型患者标本均表现为CLN5阴性,仅1例C型患者标本有CLN5的表达。成血管母细胞瘤患者瘤囊表现程度与CLN5表达程度有很强的相关性(相关系数=-0.732,P=0.016,n=11)。结论 脑内微血管内皮细胞CLN5的表达降低与小脑成血管母细胞瘤瘤囊形成有关。肿瘤发展过程中,脑微血管内皮细胞紧密连接的破坏可能是成血管母细胞瘤瘤囊形成的主要病理生理机制。 Objective To investigate the expression of claudin-5 (CLN5) in hemangioblastoma and its significance in the process of tumor formation. Methods All 11 clinical specimens of this study were obtained from patients with cerebellar hemangioblastoma without preoperative hormonal therapy and radiation therapy. Four of them were associated with von Hippel-Lindau disease. According to the preoperative imaging examination, the gross appearance of the tumor was divided into three types: S type, with solid tumors as the main performance; SC type, with larger solid tumors, accompanied by obvious tumor capsule formation; C type, The formation of a larger tumor capsule as the main manifestation of the tumor itself is only manifested as tumor wall nodules. ABC immunohistochemistry was used to detect the expression of CLN5 on the microvascular endothelial cells of the tumor tissue specimens. According to the positive rate of microvessel endothelial cells, immunohistochemistry results were divided into three grades: negative, the positive rate of microvascular endothelial cells in specimens ≤ 5%; weak positive, 5%