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一名8岁儿童的横纹肌样脑膜瘤。

Rhabdoid meningioma in an eight-year-old child.

作者信息

Morina Arsim, Kelmendi Fatos, Morina Oamile, Pazanin Leo, Dragusha Shefki, Ahmeti Feti, Morina Dugakjin

机构信息

Clinic of Neurosurgery, University Clinical Center of Kosova, Prishtina, Kosova.

出版信息

Med Arh. 2010;64(2):123-4.

Abstract

INTRODUCTION

We report a case of Rhabdoid meningioma in an eight-year-old child which is the fifth case in the world according to our knowledge.

MATERIAL AND METHOD

An 8-year-old girl was seen in our University Clinic of Neurosurgery with one month history of increasing headaches, vomiting and increasing frequency of grand mal seizures (known history of epilepsy). Her past medical history was not significant, except the epilepsy. After opening the dura mater, an extra axial tumor was found. The neoplasm invaded the brain and was highly vascular. The lesion was totally excised and 2 cm of adjacent dura materwas excised--Simpson O.

RESULTS

The postoperative course was without significant events. The patient was discharged home on the seventh postoperative day. Seizures were controlled with anticonvulsant therapy. Postoperative control head CT scan demonstrated total removal of the lesion. HP analysis: Paraffin-embedded tissue sections stained with H&E revealed meningothelial tumor with rhabdoid morphology characterized by sheets of tumor cells with eccentric nuclei, variably abundant eosinophilic cytoplasm and intracytoplasmic hyaline paranuclear inclusions. The number of mitoses was up to four in ten high-power fields, and the Ki-67 proliferation index was 4.4%. The histopathological diagnosis was rhabdoid meningioma (grade III).

DISCUSSION

Radical surgery (Simpson grade 1) has been shown to significantly enhance prognosis in atypical and malignant meningiomas.

CONCLUSION

Rhabdoid meningioma is an anaplastic, very rare subtype of malignant meningioma. The prognosis for rhabdoid meningioma depends on their proliferative activity and the possibility of radical removal.

摘要

引言

我们报告一例8岁儿童的横纹肌样脑膜瘤病例,据我们所知这是世界上第五例。

材料与方法

一名8岁女孩因头痛加剧、呕吐及癫痫大发作频率增加(有癫痫病史)1个月,就诊于我校神经外科诊所。除癫痫外,她既往病史无特殊。打开硬脑膜后,发现一个轴外肿瘤。该肿瘤侵犯脑实质且血管丰富。病变被完全切除,并切除了2厘米的相邻硬脑膜——辛普森0级。

结果

术后过程无重大事件。患者术后第7天出院。癫痫发作通过抗惊厥治疗得到控制。术后头颅CT复查显示病变已完全切除。苏木精-伊红染色分析:石蜡包埋组织切片经苏木精-伊红染色显示为脑膜内皮肿瘤,具有横纹肌样形态,其特征为成片的肿瘤细胞,核偏位,嗜酸性细胞质丰富程度不一,胞质内有透明的核旁包涵体。每10个高倍视野中核分裂数最多为4个,Ki-67增殖指数为4.4%。组织病理学诊断为横纹肌样脑膜瘤(三级)。

讨论

根治性手术(辛普森一级)已被证明可显著改善非典型和恶性脑膜瘤的预后。

结论

横纹肌样脑膜瘤是一种间变性、非常罕见的恶性脑膜瘤亚型。横纹肌样脑膜瘤的预后取决于其增殖活性和根治性切除的可能性。

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