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首页> 外文期刊>The Canadian Journal of Neurological Sciences: le Journal Canadien des Sciences Neurologiques >Recurrent PNET with MGMT methylation responds to temozolomide.
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Recurrent PNET with MGMT methylation responds to temozolomide.

机译：MGMT甲基化的复发性PNET对替莫唑胺有反应。

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摘要

Primitive neuro-ectodermal tumours (PNET) are neuro-epithelial neoplasms arising from the germinal matrix of the primitive neural tube. Primitive neuro-ectodermal tumours are predominately a tumour of childhood, accounting for about 20% of primary central nervous system (CNS) malignancies in children1. Typically PNETs occur in the posterior fossa, but supratentorial PNETs in children still account for about 2.8% of all primary tumours of childhood and adolescence2. Much is known about the management of PNETs in children. In adults, on the other hand, supratentorial PNETs are exceedingly rare, with only a handful of cases reported in the current literature. Thus, optimal medical management of supratentorial PNETs in adults remains unclear. Chemotherapy options that have been utilized in previous reports include conventional agents such as vincristine, cisplatinum, cyclophosphamide, procarbazine, and CCNU2-3, in addition to recent reports of successful treatment with temozolomide1'4. Here, we present the case of a patient with a supratentorial PNET diagnosed in adulthood, with tumour recurrence on carboplatin and etoposide, which responded to subsequent treatment with temozolomide.
机译：原始神经外胚层肿瘤（PNET）是源自原始神经管生发基质的神经上皮肿瘤。原始的神经外胚层肿瘤主要是儿童期肿瘤，约占儿童原发性中枢神经系统（CNS）恶性肿瘤的20％1。 PNETs通常发生在后颅窝，但是儿童的幕上PNETs仍然占儿童期和青春期所有原发肿瘤的约2.8％2。关于儿童中的PNET的管理众所周知。另一方面，在成年人中，幕上PNET极为罕见，在当前文献中仅报道了少数病例。因此，尚不清楚成年人的幕上PNET的最佳医疗管理。除最近成功使用替莫唑胺1'4治疗的报道外，以前的报告中已采用的化学疗法选择包括长春新碱，顺铂，环磷酰胺，丙卡巴肼和CCNU2-3等常规药物。在这里，我们介绍了一名成年后诊断为幕上PNET的患者，卡铂和依托泊苷的肿瘤复发，对替莫唑胺的后续治疗有反应。

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