Kitaz Mohammad Nour, Dabbagh Ezeddin, Hallak Hasan, Alali Kutaiba, Wereekia Mahmoud, Kadi Mohamad
Department of Neurosurgery, Aleppo University Hospital, University of Aleppo, Aleppo, Syrian Arab Republic.
Faculty of Medicine, University of Aleppo, Aleppo, Syrian Arab Republic.
Int J Surg Case Rep. 2024 Oct;123:110089. doi: 10.1016/j.ijscr.2024.110089. Epub 2024 Jul 27.
Ependymomas arise from the ependymal cells that line the brain ventricles, and central canal. In children most of them are benign. However, cortical anaplastic ependymomas are very rare in pediatrics.
A 10 months girl presented with vomits for one week, increased head circumference, psychomotor development delay, left facial nerve (VII) palsy, and left hemiparesis 3/5. Magnetic resonance imaging (MRI) of the brain demonstrated a large parenchymal lesion filling most of the right hemisphere. She underwent a total excision of the lesion. The tumor had no connection to the ventricular ependymal lining. No adjuvant chemotherapy or radiotherapy was considered. The final diagnosis is Anaplastic Ependymoma (WHO Grade III).
Cortical anaplastic ependymomas are extremely rare. In pediatrics they affect frontal, frontoparietal, and parietal lobes, Temporal and occipital lobes are uncommon. Migration disorders from the germinal matrix and the differentiation of primitive neuroectodermal tumors along the ependymal lineage are considered two hypotheses that explain the pathogenesis of ectopic ependymomas.
Ependymomas should be considered a differential diagnosis in children, as the successful total removal of ependymomas in that age saves the patient from the need of adjuvant therapy such as radio-therapy or chemotherapy.
室管膜瘤起源于衬于脑室和中央管的室管膜细胞。在儿童中,大多数室管膜瘤是良性的。然而,皮质间变性室管膜瘤在儿科非常罕见。
一名10个月大的女童出现呕吐一周,头围增大,精神运动发育迟缓,左侧面神经(VII)麻痹,左侧偏瘫肌力为3/5。脑部磁共振成像(MRI)显示一个大的实质性病变,占据了大部分右半球。她接受了病变的全切除。肿瘤与脑室室管膜衬里无连接。未考虑辅助化疗或放疗。最终诊断为间变性室管膜瘤(世界卫生组织III级)。
皮质间变性室管膜瘤极为罕见。在儿科,它们累及额叶、额顶叶和顶叶,颞叶和枕叶较少见。源自生发基质的迁移障碍以及原始神经外胚层肿瘤沿室管膜谱系的分化被认为是解释异位室管膜瘤发病机制的两种假说。
室管膜瘤应被视为儿童疾病的鉴别诊断之一,因为在该年龄段成功全切除室管膜瘤可使患者无需接受放疗或化疗等辅助治疗。