Trivedi Darshan, Xiong Zhenggang
Department of Pathology and Laboratory Medicine, Tulane University School of Medicine, New Orleans, LA, USA.
Intractable Rare Dis Res. 2017 May;6(2):128-131. doi: 10.5582/irdr.2017.01015.
A 7-month-old boy presented with gastrointestinal disturbance, mild neurologic deficit of the left lower extremity and levo-scoliosis of the thoracic spine. Magnetic resonance imaging demonstrated a large intramedullary lesion involving the thoracic spine, from level T1 to T11. Histologic analysis showed a glial tumor with fibrillary processes arranged in radial pattern around mucoid fibrovascular cores with a high proliferative index (focally up to 80%) and prominent vascular endothelial hyperplasia. These findings were consistent with an anaplastic myxopapillary ependymoma. Subtotal resection was performed a T3-T10 laminoplasty. A ventricular shunt was placed, and the patient subsequently received chemoradiation therapy. To date, this is the second case of a myxopapillary ependymoma with high-grade anaplastic features and the first case in an infant reported in the literature.
一名7个月大的男婴出现胃肠道紊乱、左下肢轻度神经功能缺损和胸椎左旋脊柱侧弯。磁共振成像显示一个累及胸椎的大髓内病变,从T1至T11水平。组织学分析显示为胶质肿瘤,其纤维状突起围绕黏液样纤维血管核心呈放射状排列,增殖指数高(局部高达80%)且有明显的血管内皮细胞增生。这些发现符合间变性黏液乳头型室管膜瘤。通过T3 - T10椎板成形术进行了次全切除。放置了脑室分流管,患者随后接受了放化疗。迄今为止,这是第二例具有高级别间变特征的黏液乳头型室管膜瘤病例,也是文献报道的首例婴儿病例。