Milena Laure-Kamionowska, MD, PhD, Department of Experimental and Clinical Neuropathology, Mossakowski Medical Research Centre, Polish Academy of Sciences, 5 Pawinskiego St., 02-106 Warsaw, Poland, e-mail:
Folia Neuropathol. 2013;51(4):333-9. doi: 10.5114/fn.2013.39724.
Congenital central nervous tumours form a unique group of neoplasms. They are different from other tumour groups not only due to the onset time but also to their histopathology, anatomic location, and biologic behaviour. Congenital glioblastoma is one of the rarest types of congenital brain tumours and is uncommon in the prenatal period. We report a rare case of congenital glioblastoma detected prenatally by ultrasound examination and magnetic resonance imaging at 26 gestational weeks. Based on MRI findings and consultation of a team of specialists, pregnancy was terminated at 28 weeks. The newborn presented hydrops foetal. The child died shortly after birth due to cardiorespiratory insufficiency. At autopsy a large tumour with a spongy-like appearance was found. The tumour involved nearly the whole right cerebral hemisphere and led to marked hydrocephalus. In the histological and immunohistochemical examination, the tumour presented features of glioblastoma. Neoplastic cells were immunopositive for GFAP, S-100 protein and negative for neuronal markers. Frequent mitoses and high MIB-1 labelling index were seen in the tumour areas. The coexistence of tumour and vascular developmental anomaly was stated. The conglomerates of numerous, distended, thin-walled foetal-like blood vessels were located beside the tumour tissue, which presented disturbance in differentiation and maturation of the vascular net. Such coexistence of malignant glioma with vascular developmental anomaly is unique.
先天性中枢神经系统肿瘤形成了一组独特的肿瘤。它们不仅由于发病时间，还由于其组织病理学、解剖位置和生物学行为与其他肿瘤群不同。先天性成胶质细胞瘤是最罕见的先天性脑肿瘤类型之一，在产前并不常见。我们报告了一例罕见的先天性成胶质细胞瘤病例，该病例在 26 孕周时通过超声检查和磁共振成像被产前发现。根据 MRI 发现，并咨询专家团队的意见，妊娠在 28 孕周终止。新生儿出现胎儿水肿。由于心肺功能不全，孩子出生后不久就死亡了。尸检时发现一个呈海绵状外观的大型肿瘤。肿瘤几乎累及整个右大脑半球，导致明显的脑积水。在组织学和免疫组织化学检查中，肿瘤呈现出成胶质细胞瘤的特征。肿瘤细胞免疫阳性表达 GFAP、S-100 蛋白，而神经元标志物阴性。肿瘤区域可见频繁的有丝分裂和高 MIB-1 标记指数。还指出了肿瘤和血管发育异常的共存。大量扩张的薄壁胎儿样血管聚集体位于肿瘤组织旁边，这表明血管网的分化和成熟受到干扰。这种恶性胶质瘤与血管发育异常的共存是独特的。
