北京大学学报(医学版) ›› 2017, Vol. 49 ›› Issue (5): 904-909. doi: 10.3969/j.issn.1671-167X.2017.05.029
王惠1, 2, 叶锦棠3, 姚红新4, 李东1, 董颖1
WANG Hui1, 2, YE Jin-tang3, YAO Hong-xin4, LI Dong1, DONG Ying1
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。
中图分类号:
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