脊髓血管母细胞瘤5例临床病理分析

Spinal Cord Haemangioblastoma：A Clinicopathological Analysis of 5 Cases

目的探讨脊髓血管母细胞瘤（Haemangioblastoma，HB）的临床、病理学特点，诊断及鉴别诊断。方法回顾分析5例脊髓HB的临床资料、病理形态和免疫组织化学染色结果，并复习相关文献。结果5例病例中发生于胸椎1例、腰椎2例、颈椎3例（其中1例多发），HE染色镜下形态基本相似，HB主要由两种成分构成：空泡状大间质细胞和丰富的毛细血管网。免疫病理显示：间质细胞EGFR、NSE均阳性，血管内皮细胞CD34、CD31阳性，GFAP、CK、EMA、Ki-67均阴性。结论中枢神经系统HB是一种良性肿瘤，脊髓少见，根据组织学特征及免疫组织化学检测可做出明确诊断，免疫标记对其鉴别诊断有帮助。

Objective To explore the clinicopathological features of spinal cord haemangioblastoma and its diagnosis and differential diagnosis.Methods The clinical features,histopathology and immunophenotype of five cases of spinal cord haemangioblastoma were analyzed retrospectively.Results Among five cases,one case occurred in thoracic spinal cord,two cases in lumbar spinal cord and three cases in cervical spinal cord.Histologically, haem- angioblastoma is composed of large pleomorphic stromal cells and capillary network.Immunohistochemically, the endothelial cells of haemangioblastoma are usually stained with CD34 and CD31,whereas stromal cells are negative.Stromal cells are usually stained positive with EGFR and NSE,meanwhile they are negative for GFAP, CK,EMA and Ki-67.Conclusions Haemangioblastomas are benign neoplasms that originate in the central nervous system.Spinal cord haemangioblastomas are tmcommon.A correct diagnosis ofhaemangioblastoma can be made by combining histology with immunophenotype,and immunohistochemistry may be helpful for differential diagnosis.