婴儿胚胎发育不良性神经上皮肿瘤1例报道并文献复习

发布时间：2018-05-04 14:13

  本文选题：脑肿瘤 + 肿瘤　； 参考：《北京大学学报(医学版)》2017年05期

【摘要】：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。
[Abstract]:This paper reports a case of dysembryoplastic neuroepithelial tumor (DNT) of left temporal lobe embryogenesis in infants. The clinical and pathological features, imaging features, surgical treatment and prognosis of DNTs were introduced, and the diagnosis of the disease was reviewed. Differential diagnosis and treatment and prognosis were analyzed to improve the clinical and pathologist understanding of this rare disease. The patient was 11 months old and had early onset. The clinical manifestation was partial epileptic seizure, but the drug response was good. The left temporal lobe occupying and suspected astrocytoma were demonstrated by imaging, but some of them still had the triangle characteristic of DNT. In this case, the lesion was small in size, and there were not only typical glial neuron structure-myxoid columnar structure, but also some atypical regions. There was no cortical dysplasia in the surrounding brain parenchyma, and no mutation of BRAFV600E locus was detected. Epilepsy. DNT is a rare neuroepithelial tumor with good prognosis 6 months after total resection of tumor. The infantile patients have their own characteristics. Complete resection of tumor is effective without radiotherapy or chemotherapeutic therapy.
【作者单位】： 北京大学第一医院病理科;北京大学第一医院电镜室;北京大学第一医院影像科;北京大学第一医院小儿外科;
【分类号】：R739.4
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本文编号：1843270