小脑血管母细胞瘤2例临床病理分析

目的:探讨小脑血管母细胞瘤（HB)临床影像学表现、病理学特征及免疫组化特点，为鉴别诊断的准确率提供依据。方法:回顾性分析2015年10月至2017年4月诊治的2例HB患者临床资料、影像学表现、病理学特征及免疫组化特点并复习相关文献。结果:2例HB患者均以头痛为首发症状，影像学检查提示小脑半球囊实性和实质性占位，镜下肿瘤主要以大间质细胞和丰富的毛细血管网构成，并根据间质细胞的丰富程度分为细胞亚型，其肿瘤组织内含大量的间质细胞，细胞呈圆形或多角形，胞浆丰满呈空泡状；网状亚型，肿瘤组织内含大量的毛细血管，网织染色示有丰富的网状纤维。HB间质细胞中Vimentin、S-100蛋白、Inhibin-a、CD56表达均阳性且NSE、VEGF高表达，EMA、CD34、CD31、GFAP、EGFR均呈阴性。血管内皮细胞均表达内皮细胞标记物CD34、CD31及FⅧAg。Ki-67增殖指数标记阳性率＜5％。结论:HB是临床较为少见的中枢神经系统良性肿瘤（WHO Ⅰ级)，影像学表现具有一定的特异性，结合病理学特征及免疫组化特点，有助于提高诊断的准确率。

A clinicopathological analysis of 2 cases of erebellum hemangioblastoma

Objective:To investigate the clinical imaging findings，pathology feature and immuno histochemistry characteristics of cerebellum hemangioblastoma（HB) and to provide evidence for the accuracy of differential diagnosis.Methods:Retrospectively analyse the clinical data of 2 patients with HB from October 2015 to April 2017 in imaging findings，histopathological features and immunohistochemistry，pathology feature and relevant literature was reviewed.Results:Headache was the first symptom in all patients with HB，and imaging examinations revealed solid cystic and substantial tumor occupancy in the cerebellar hemisphere，microscopically tumors consisted of large interstitial cells and a rich network of capillary vessels，and they were classified into cell subtypes according to the abundance of interstitial cells.The cells were round or polygonal，and the cytoplasm was full showed vacuole-shape.For reticulon subtype，tumor tissue contained a large number of capillaries，reticular fiber staining showed rich reticular fibers.The HB interstitial cells were positive for Vimentin，S-100 protein，Inhibin-a and CD56，and were highly expressed NSE and VEGF.EMA，CD34，CD31，EGFR and GFAP were negative.Vascular endothelial cell expressed endothelial markers CD34，CD31 and FVⅢAg.The positive rate of Ki-67 proliferation index was<5%.Conclusion: HB is a clinically rare benign tumor of the central nervous system（WHO level Ⅰ).Imaging performance has a certain degree of specificity.Combined with pathological features and immunohistochemical features，it contributes to the accuracy of diagnosis.