Mørk S J, Rubinstein L J
Cancer. 1985 Apr 1;55(7):1536-42. doi: 10.1002/1097-0142(19850401)55:7<1536::aid-cncr2820550721>3.0.co;2-a.
This article reviews the clinicopathologic features of 12 ependymoblastomas, including those of 7 previously unreported cases. The histologic characteristics included a high density of small to medium-sized neuroepithelial cells with a uniform cytologic appearance, frequent mitotic figures, and numerous diagnostic ependymal rosettes and tubules. Differentiation was restricted to glial precursor cells and to cells with the differentiating features of ependymal cells. Cytogenetically, the tumor cells with the differentiating hallmarks of ependymal cells but which have retained their mitotic activity were considered to be ependymoblasts. Many of the rosettes in the tumors were of the ependymoblastic type, but ependymal rosettes were also present. The absence of pleomorphism, giant cells, multinucleation and pseudopalisades, and the scanty proliferation of vascular endothelial cells are additional features that delineate this tumor from an anaplastic (malignant) ependymoma. The median age of the patients was 2 years. After surgical treatment the median survival time was 12 months. Because of the frequency of leptomeningeal involvement, whole neuraxis radiation should be considered.
本文回顾了12例室管膜母细胞瘤的临床病理特征，包括7例既往未报道病例的特征。组织学特征包括中小神经上皮细胞密度高，细胞外观一致，有频繁的有丝分裂象，以及大量典型的室管膜菊形团和小管。分化仅限于神经胶质前体细胞和具有室管膜细胞分化特征的细胞。细胞遗传学上，具有室管膜细胞分化特征但保留有丝分裂活性的肿瘤细胞被认为是室管膜母细胞。肿瘤中的许多菊形团是室管膜母细胞型的，但也存在室管膜菊形团。缺乏多形性、巨细胞、多核和假栅栏状结构，以及血管内皮细胞增殖稀少，是将该肿瘤与间变性（恶性）室管膜瘤区分开来的其他特征。患者的中位年龄为2岁。手术治疗后的中位生存时间为12个月。由于软脑膜受累频繁，应考虑全脑脊髓放疗。
