星形母细胞瘤临床病理观察

Clinicopathologic features of astroblastoma: a case report and review of literatures

目的探讨星形母细胞瘤临床病理特点、病理诊断与鉴别诊断、免疫组化及电镜特点。方法对1例高级别星形母细胞瘤进行临床病理学分析及文献复习。结果患儿女性,5岁。影像学特点为左颞顶叶边界清楚的囊实性结节。组织学示肿瘤细胞伴短粗胞质突起,放射状围绕在血管周围呈假菊形团样排列,瘤细胞异型性显著,瘤巨细胞及核分裂象可见,易见血管壁玻璃样变性。免疫组化示GFAP和vimentin(+)。电镜下肿瘤细胞表面见微绒毛突起。完全手术切除后未行放、化疗,术后随访22个月无复发。结论星形母细胞瘤是罕见、来源未明的神经上皮肿瘤。组织学可分为低级别和高级别两类,高级别星形母细胞瘤预后比低级别星形母细胞瘤差,手术完全切除肿块是主要治疗方法,所有病例均应密切随访。

Purpose To explore the clinicopathological characteristics, pathological diagnosis and differential diagnosis, and the immunohistochemical and uhrastruetural features of astroblastoma. Methods One case of high grade astroblastoma was reported, with review of relevant literature. Results A well-demarcated mass with both solid and cystic components was found in left temporalparietal lobe on a brain CT and MRI. Histological features were characterized by a typical perivascular orientation of tumor cells with broad, short processes resembling pseudorosettes. Marked cellular atypia, giant tumor cell, high mitotic rate, significant sclerosis of the vascular walls without endothelial proliferation could easily be seen compatible with high grade astroblastoma. Immunohistochemically, the tumor cells showed positivity for GFAP,Vim. Microvillous projections were observed on the tumour ceil surface by electron microscopy. The patient underwent gross-totally-resected eraniotomy without postoperative radiotherapy and chemotherapy. She was followed-up for 22 months and no recurrence was detected. Conclusions Astroblastomas are distinct and rare neuroepithelial tumors of uncertain origin. Astroblastomas are classified into two distinct histological types：low grade and high grade. The so-called high-grade astroblastomas are thought to have worse prognosis than those with low grade histological features. Gross total resection is reported to be the best way of treating an astroblastoma. Close follow-up of all cases are recommended.