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[大脑半球多灶性胶质瘤伴冯·雷克林霍增氏病:病例报告]

[Multifocal gliomas in cerebral hemisphere associated with von Recklinghausen's disease: case report].

作者信息

Mukai K, Kitamura K, Asano N, Ohshima T, Hondo H, Matsumoto K

机构信息

Department of Neurological Surgery, School of Medicine, University of Tokushima.

出版信息

No Shinkei Geka. 1989 Feb;17(2):197-202.

PMID:2499819
Abstract

A case of intracerebral multifocal gliomas with von Recklinghausen's disease is reported. A 12-year-old boy was admitted to our hospital with an episode of convulsive attacks which were uncontrolled by anticonvulsants. CT scan and MRI revealed small well circumscribed tumors in the left frontal cortex and left parietal subcortex. Both of the tumors revealed low density in plain CT scan and low intensity in T1 weighted MRI. The vascularity of these tumors was poor in cerebral angiography. In other words these tumors were of a similar nature. The histology of the frontal tumor, which was totally removed surgically, showed typical pilocytic astrocytoma. The other tumor in the left parietal subcortex must also be included in the category of low grade glioma. In the sixth month after the operation, we could find neither recurrence of the frontal glioma nor enlargement of the parietal tumor, on CT and MRI findings. Immunohistochemically, the outer portion of the Rosenthal fiber in this tumor was positive for GFAP and S-100 protein, but the inner portion was negative, because the GFAP and S-100 protein there had degenerated. The cytoplasm of this tumor's cell was abundant with mitochondria and Golgi's bodies compared to the fibrillary astrocytoma. This case may be the first case of multifocal gliomas in the same cerebral hemisphere. We suggest that multifocal gliomas grow naturally, and over the years, tumors combine with each other and finally constitute a large type diffuse glioma.

摘要

报告了一例患有冯·雷克林霍增氏病的脑内多灶性胶质瘤病例。一名12岁男孩因惊厥发作入院,抗惊厥药物无法控制病情。CT扫描和MRI显示左额叶皮质和左顶叶皮质下有边界清晰的小肿瘤。在平扫CT扫描中,这两个肿瘤均显示低密度,在T1加权MRI中显示低信号。脑血管造影显示这些肿瘤血管较少。换句话说,这些肿瘤性质相似。手术完全切除的额叶肿瘤组织学检查显示为典型的毛细胞型星形细胞瘤。左顶叶皮质下的另一个肿瘤也必须归类为低级别胶质瘤。术后六个月,CT和MRI检查未发现额叶胶质瘤复发或顶叶肿瘤增大。免疫组织化学检查显示,该肿瘤中罗斯enthal纤维的外层GFAP和S-100蛋白呈阳性,但内层为阴性,因为那里的GFAP和S-100蛋白已经退化。与纤维型星形细胞瘤相比,该肿瘤细胞的细胞质富含线粒体和高尔基体。该病例可能是同一脑半球多灶性胶质瘤的首例。我们认为多灶性胶质瘤自然生长,多年来,肿瘤相互融合,最终形成大型弥漫性胶质瘤。

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