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[一例小脑神经母细胞瘤]

[A case of cerebellar neuroblastoma].

作者信息

Ibayashi N, Ueda S, Uchibori M, Hirakawa K

出版信息

No Shinkei Geka. 1987 Apr;15(4):459-63.

PMID:3614540
Abstract

A three-year-old boy with a progressive history of headache, vomiting and ataxia in the course of 2 months, was admitted on August 1983, when he was lethargic. Neurological examination revealed dysphagia, scanning speech and tremor in the bilateral hand. CT scan showed a very large enhanced mass in the center of posterior fossa with central necrosis in it and the dilatation of whole ventricular system. Suboccipital craniectomy was immediately performed and the tumor that occupied the vermis and invaded into both cerebellar hemisphere was subtotally removed. Postoperative irradiation was well performed: 4140 rads to the whole brain and 3162 rads to the spinal cord. However, 5 months later, facial palsy in the left side and progressive ataxia became prominent. CT scan showed multiple enhanced masses in the left trigonum and right anterior horn of the lateral ventricles and in the left cerebellopontine angle. In spite of chemotherapy, the patient had a down-hill course, especially after the ventricular hemorrhage, and died on June 9th, 1984. Histologically, the tumor had a lobulated appearance with an aggregation of tumor cells encircled by vascular septae. The cells within lobules generally had vesicular nuclei, which were arranged in parallel row. Occasionally smaller hyperchromatic cells with scant cytoplasm were present along the vascular septae. Reticulin was present within the septa, but was not observed within the lobules. Scattered astrocytic cells and processes were identified within the lobules by the immunoperoxidase technique for GFAP. The fibrillary cytoplasmic processes within the lobules were stained by immunoperoxidase technique for neurofilament (68K).(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

一名三岁男孩,在两个月内逐渐出现头痛、呕吐和共济失调症状,于1983年8月入院,当时他处于嗜睡状态。神经系统检查发现吞咽困难、吟诗样言语和双手震颤。CT扫描显示后颅窝中央有一个非常大的强化肿块,中央有坏死,整个脑室系统扩张。立即进行了枕下颅骨切除术,部分切除了占据蚓部并侵犯双侧小脑半球的肿瘤。术后进行了良好的放疗:全脑照射4140拉德,脊髓照射3162拉德。然而,5个月后,左侧面瘫和进行性共济失调变得明显。CT扫描显示左侧三角区、右侧侧脑室前角和左侧小脑桥脑角有多个强化肿块。尽管进行了化疗,患者病情仍逐渐恶化,尤其是在脑室出血后,于1984年6月9日死亡。组织学上,肿瘤呈分叶状外观,肿瘤细胞聚集,被血管间隔包围。小叶内的细胞通常有泡状核,排列成平行行。偶尔,沿着血管间隔会出现较小的深染细胞,细胞质稀少。间隔内有网状纤维,但小叶内未观察到。通过GFAP免疫过氧化物酶技术在小叶内鉴定出散在的星形细胞和突起。小叶内的纤维状细胞质突起通过神经丝(68K)免疫过氧化物酶技术染色。(摘要截断于250字)

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