Otsuki T, Takahashi S, Sonobe M, Kubota Y, Shibazaki S
No Shinkei Geka. 1981 Dec;9(13):1535-40.
Primary cerebral neuroblastoma is rare. The case reported here was a 2-year-old girl who was comatose on admission to the Mito National Hospital. She had experienced headache and vomiting during the last two months. Neurological examination showed bilateral papilledema, left oculomotor palsy and bilateral Babinski's sign. A-P view of the left carotid angiogram showed square shift of the anterior cerebral artery to the right, and the lateral view showed upward and anterior displacement of the middle cerebral artery. CT revealed large mass lesion in the left parieto-temporal lobe, which was slightly enhanced on the contrast study. Subtotal removal of the tumor was performed on the day of admission. On the 15th postoperative day, she was started on radiation therapy to the brain and received 1900 rads. Vincristin and ACNU were administrated with radiation therapy. She improved progressively five weeks after the operation and CT demonstrated marked shrinkage of the tumor. However, signs of increased intracranial pressure were reappeared and she died six months after the operation. Autopsy showed well defined soft and grayish white tumor in the occipito-temporal lobe, which extended to the left thalamus and basal ganglia. No other tumor was present extracranially. Microscopic examination demonstrated Homer-Wright rosette and zonal filament. Pathological diagnosis was neuroblastoma.
原发性脑成神经细胞瘤很罕见。本文报道的病例是一名2岁女孩,入院时昏迷,水户市立医院。她在过去两个月里出现头痛和呕吐。神经系统检查显示双侧视乳头水肿、左侧动眼神经麻痹和双侧巴宾斯基征。左颈动脉血管造影正位片显示大脑前动脉向右方移位呈方形,侧位片显示大脑中动脉向上向前移位。CT显示左侧顶颞叶有巨大肿块病变,增强扫描时有轻度强化。入院当天行肿瘤次全切除术。术后第15天开始对脑部进行放射治疗,共接受1900拉德。放疗期间给予长春新碱和嘧啶亚硝脲。术后五周病情逐渐好转,CT显示肿瘤明显缩小。然而,颅内压升高的体征再次出现,术后六个月死亡。尸检显示枕颞叶有边界清楚的软灰白色肿瘤,延伸至左侧丘脑和基底神经节。颅外未发现其他肿瘤。显微镜检查显示霍纳-赖特菊形团和带状细丝。病理诊断为成神经细胞瘤。