先天性室管膜母细胞瘤尸检分析并文献复习

Congenital ependymoblastoma: A neonatal autopsy case and review of literature

目的探讨先天性室管膜母细胞瘤的临床及组织病理学特征。方法对1例经尸检诊断的先天性室管膜母细胞瘤的组织病理学、免疫组织化学、超微结构以及DNA倍体情况进行研究,并结合文献复习。结果孕中经B超发现的颅内肿瘤,经尸检确定位于左颞叶近侧脑室后角处,直径约3.5 cm。肿瘤细胞胞核致密浓染,胞质少,密集成团形成具有复层细胞和近腔处活跃核分裂特征的菊形团。免疫组化结果示肿瘤细胞vim entin阳性,GFAP少数阳性。结论室管膜母细胞瘤是一种极少见的神经上皮胚胎性肿瘤,具有特征性的"室管膜母细胞瘤"菊形团结构。肿瘤主要位于幕上,好发于婴幼儿,具有高度侵袭性,预后极差。

Purpose To study the clinical and histopathological features of the congenital ependymoblastoma. Methods An autopsy case of congenital ependymoblastoma was studied by light microscopy, immunohistochemistry, transmission electron microscopy and flow cytometry, with a review of the relevant literature. Results The tumor revealed by ultrasonic examination during pregnancy, located in the left temporal lobe in autopsy ,with a diameter of about 3.5 cm, adjacent to the posterior fossa of lateral ventricle. The tumor cells contained compactly basophilic nuclei, scanty cytoplasm, densely packing into multilayered rosettes with numerous mitotic figures adjacent to lumen. Immunohistochemical staining showed the positive immunoreactive for vimentin and GFAP （minority）. Conclusions The epondymoblastoma is a rare type of embryonal neuroepithelial tumor, with a hallmark of ependymoblastomatous rosette. The majority arises in supratentorial locations, developing during the newborn and infancy, It has an aggressive pattern of biological behavior with high metastatic potential and very poor prognosis.