颅内血管外皮细胞瘤42例临床病理分析

Clinicopathologic analysis of 42 cases of intracranial hemangiopericytoma

目的探讨颅内血管外皮细胞瘤病理组织学特征及影像学表现,分析其诊断要点。方法回顾性分析42例颅内血管外皮细胞瘤临床病理特征、免疫表型、影像学特点、诊断及鉴别诊断。结果颅内血管外皮细胞瘤临床表现主要为头晕、头痛、视力下降;CT扫描和MRI影像检查示颅内与脑膜有关的占位性病变,信号多不均匀,并有显著不均匀强化;肿瘤组织学显示瘤细胞弥漫分布,异型性不明显,血管明显增生,为典型的薄壁鹿角状血管;免疫组化显示CD34、CD99和Vimentin均呈较强阳性;Ki-67表达较高,阳性细胞数平均为15.4%,而其他标记物的表达不尽相同。结论颅内血管外皮细胞瘤来源于毛细血管的Zimmerman细胞,组织形态具有一定的特征性,在颅内主要表现为恶性肿瘤的特征,预后不良。颅内血管外皮细胞瘤须注意与脑膜瘤、胶质瘤、神经鞘瘤等相鉴别。

Objective To discuss the histopathological features and imaging findings of intracranial hemangiopericytoma and the essential points of diagnosis. Methods The clinicopathologic features, immunophenotype, imaging character, diagnosis and differential diagnosis of 4 2 cases with intracranial hemangiopericytoma were studied retrospectively. Results The major chnical manifestations of intracranial hemangiopericytoma included dizziness, headache and vision impairment. CT scan and MRI showed intracranial occupying lesion related to meninges, and most of the tumors showed inhomogeneous signals and marked asymmetric enhancement. Histology demonstrated that the tumor cells were diffuse without obvious atypia, significant vascular proliferation with typical ramiform thin- walled blood vessels. Immunohistochemistry showed that most of the tumor cells were strongly positive for CD 3 4 , CD 9 9 and vimentin ; Ki- 6 7 was highly expressed with averaged positive cells of 15.4 % , which was much different from the expression of other markers. Conclusion Intracranial hemangioper/cytoma originates from capillary ＇ s Zimmerman cell, with certain histological characteristics of malignant tumor and the prognosis is poor. This tumor should be differentially diagnosed from meningioma, glioma, schwannoma and so on.