Dysembryoplastic Neuroepithelial Tumor (Dnet) in a Young Female: A Rare Case Presentation

Sarah Irfan, Mazhar Fahim, S. Rahman, K. Akhtar
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引用次数: 0

Abstract

Dysembryoplastic neuroepithelial tumor (DNET) is a recently described, morphologically unique, and surgically curable low grade brain tumor which is included in the WHO CNS classification as neuronal and mixed neuronal glial tumor. It is usually seen in children and young adults. This entity first described by Damas-Duport in 1998 is usually non-recurring with an excellent prognosis. We present a case of 30 year old female experiencing seizures with well defined space occupying lesion in the frontal region of brain. Contrast enhanced Magnetic Resonance Imaging was done which revealed well defined multilobulated solid cystic mass lesion in cortical/ subcortical region of left frontal lobe. Histological features suggested dysembryoplastic neuroepithelial tumor which was subsequently confirmed on immunohistochemistry by GFAP and S-100 positivity. The principal differential diagnosis is oligodendrogliomas and gangliogliomas. Patient did not receive any adjuvant therapy post operatively and showed no signs of residual disease or recurrence on magnetic resonance imaging during follow up. Patient is on phenytoin for prophylaxis of seizures.
一例年轻女性胚胎发育异常神经上皮肿瘤(Dnet):一例罕见病例报告
胚胎发育异常神经上皮肿瘤(DNET)是最近发现的一种形态独特、手术可治愈的低级别脑肿瘤,被WHO CNS分类为神经元性和混合神经胶质性肿瘤。它通常见于儿童和年轻人。Damas-Duport于1998年首次描述了这种实体,通常不会复发,预后良好。我们报告一例30岁女性癫痫发作与明确的占位性病变在大脑额区。磁共振增强成像显示左侧额叶皮质/皮质下区多叶状实性囊性肿块。组织学特征提示胚胎发育异常的神经上皮肿瘤,免疫组化GFAP和S-100阳性证实。主要的鉴别诊断是少突胶质细胞瘤和神经节胶质细胞瘤。患者术后未接受任何辅助治疗,随访期间磁共振成像未见病灶残留及复发迹象。病人服用苯妥英以预防癫痫发作。
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