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背景和目的中枢神经系统血管母细胞瘤相对少见。本文收集了66例中枢血管母细胞瘤,总结其病理特征及与临床的联系。方法对66例中枢血管母细胞瘤进行回顾性研究。其诊断均经病理证实,临床资料来自病历。结果66例中男42例,女24例,男女=1.751。年龄2岁~80岁,平均年龄40.58岁。肿瘤单发者56例,多发者10例。肿瘤位于小脑者37例,脊髓13例,脑干12例,鞍区3例,桥小脑角3例,侧脑室与脑室交界1例。其中3例多发肿瘤位于上述两个不同部位。免疫组化显示肿瘤间质细胞阳性率为Vim5/5,EMA2/17(弱+),CK0/3,NSE10/12,EGFR0/4,InhibinA5/9,melanA3/5,GFAP3/15,CD340/13(血管全部着色)。结论中枢神经系统血管母细胞瘤为相对少见肿瘤,组织起源不确定。因没有相对特异的标记物,与其它形态学相似的肿瘤鉴别可借助一系列免疫组织化学组合。
Background and Objective Central nervous system hemangioblastoma is relatively rare. This article collected 66 cases of central hemangioblastoma, summarizes its pathological features and clinical connection. Methods 66 cases of central hemangioblastoma were retrospectively studied. The diagnosis was confirmed by pathology, clinical data from the medical records. Results 66 cases of male 42 cases, 24 females, male = 1.751. Aged 2 years old to 80 years old, with an average age of 40.58 years. 56 patients with single tumor, multiple in 10 cases. Tumors in the cerebellum in 37 cases, 13 cases of spinal cord, brain stem in 12 cases, 3 cases of saddle, cerebellopontine angle in 3 cases, lateral ventricle and ventricular junction in 1 case. Three cases of multiple tumors located in these two different parts. Immunohistochemistry showed that the positive rates of stromal cells were Vim5 / 5, EMA2 / 17 (weak +), CK0 / 3, NSE10 / 12, EGFR0 / 4, InhibinA5 / 9, melanA3 / 5, GFAP3 / 15, CD340 / 13 (Blood vessels all colored). Conclusion Central nervous system hemangioblastoma is a relatively rare tumor, the origin of which is uncertain. Because of the absence of a relatively specific marker, tumor identification similar to other morphologies can be based on a series of immunohistochemical combinations.