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1型神经纤维瘤病合并额叶囊性胶质母细胞瘤患者的病例报告。

Case report on a patient with neurofibromatosis type 1 and a frontal cystic glioblastoma.

作者信息

Hakan Tayfun, Aker Fugen Vardar

机构信息

Acibadem Gomec Sokak A Blok 27/11, Kadikoy, 34718 Istanbul, Turkey.

出版信息

Neurol Neurochir Pol. 2008 Jul-Aug;42(4):362-5.

Abstract

Neurofibromatosis type 1 (NF1) is one of the most common dominantly inherited disorders. Astrocytomas, especially low-grade optic nerve tumours, are frequently harboured in these patients. In this paper, a case of a lobar cystic glioblastoma and NF1 in a 28-year-old woman is presented. This patient underwent a resection of the glioblastoma, followed a multimodal therapy including radiotherapy and chemotherapy, and survived 41 months. Neurofibromatosis is a multifaceted disease in which primary malignant CNS tumours, such as glioblastoma, can be identified. Glioblastomas in these patients should be managed like the usual ones. They may benefit from treatment with temozolomide, as can GBM patients without NF, thus potentially increasing the patient's overall survival.

摘要

1型神经纤维瘤病(NF1)是最常见的显性遗传性疾病之一。星形细胞瘤,尤其是低级别的视神经肿瘤,在这些患者中很常见。本文介绍了一名28岁女性患有叶状囊性胶质母细胞瘤和NF1的病例。该患者接受了胶质母细胞瘤切除术,随后进行了包括放疗和化疗在内的多模式治疗,并存活了41个月。神经纤维瘤病是一种多方面的疾病,其中可以识别出原发性恶性中枢神经系统肿瘤,如胶质母细胞瘤。这些患者中的胶质母细胞瘤应像普通胶质母细胞瘤一样进行治疗。他们可能从替莫唑胺治疗中获益,无NF的胶质母细胞瘤患者也如此,从而有可能提高患者的总生存率。

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