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报告1例婴儿左颞叶胚胎发育不良性神经上皮肿瘤(dysembryoplastic neuroepithelial tumor,DNT)病例,介绍其临床病理特征、影像学特点、手术治疗及预后,并复习国内外文献报道,对该病的诊断、鉴别诊断以及治疗预后进行综合分析,以提高临床及病理医生对这一少见疾病的认识。本例发病年龄早,患儿仅11个月大,临床表现为部分性癫痫发作,但药物反应较好,影像学提示左颞叶占位并怀疑星形细胞瘤,但部分层面仍具有多见于DNT的三角形特征。本例病灶体积小,组织形态上不仅存在典型的特殊胶质神经元结构-黏液柱状结构,还存在一些非典型区域,周围脑实质不伴有皮层发育不良,未检测到BRAFV600E位点突变,手术全切肿瘤6个月后癫痫无发作。DNT是一种少见、预后良好的神经上皮肿瘤,婴儿期发病患者有其自身的特点。手术完整切除肿瘤效果良好,无需放射治疗或化学药物治疗。
A case of dysembryoplastic neuroepithelial tumor (DNT) was reported in 1 infant. The clinical and pathological characteristics, imaging features, surgical treatment and prognosis were reviewed. The reports of both domestic and foreign literature were reviewed and the diagnosis of the disease , Differential diagnosis and prognosis of a comprehensive analysis to improve clinical and pathological doctors on this rare disease awareness. The age of onset of this case as early as 11 months old children, the clinical manifestations of partial seizures, but the drug response is good, imaging prompted the left temporal lobe occupying and suspected astrocytoma, but some levels are still more common in Triangle features of DNT. This case of small lesions, histological morphology not only exist typical special glial neuron structure - mucoid columnar structure, there are still some atypical regions, surrounding brain parenchyma is not associated with cortical dysplasia, BRAFV600E site mutation was not detected, surgery Complete resection of the tumor 6 months after seizures no seizures. DNT is a rare, well-prognosis neuroepithelial tumor that has its own characteristics in infancy. Surgical complete resection of the tumor well, without radiotherapy or chemotherapy.