Ependymomas

P. Klimo, N. Shimony
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Abstract

Pediatric posterior fossa tumors are usually ependymoma, pilocytic astrocytoma, or medulloblastoma. Ependymoma appears well-demarcated with heterogeneous enhancement on magnetic resonance imaging (MRI). Full neural axis MRI is indicated to assess for metastatic disease. Management is typically surgical resection of the tumor, with consideration for cerebrospinal fluid diversion if patients present with severe hydrocephalus. Extent of resection of the tumor is the most important factor in predicting recurrence and overall survival, and gross total resection is ideal. Infratentorial ependymomas have 2 molecular subtypes, which has implications for responsiveness to adjuvant therapy and prognosis. Infratentorial ependymomas are biologically different from supratentorial ependymomas. Postoperative radiation improves local control.
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小儿后窝肿瘤通常为室管膜瘤、毛细胞星形细胞瘤或髓母细胞瘤。室管膜瘤在磁共振成像(MRI)上表现为界限清晰且不均匀增强。全神经轴MRI用于评估转移性疾病。治疗方法通常是手术切除肿瘤,如果患者出现严重的脑积水,考虑脑脊液转移。肿瘤的切除程度是预测复发和总生存的最重要因素,而大体全切除是理想的。幕下室管膜瘤有两种分子亚型,这对辅助治疗的反应性和预后有影响。幕下室管膜瘤在生物学上不同于幕上室管膜瘤。术后放疗改善局部控制。
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