非典型部位胚胎发育不良性神经上皮肿瘤的诊断与治疗

【摘要】目的探讨非典型部位胚胎发育不良性神经上皮肿瘤（DNT）的临床特点，提高对非典型部位DNT的诊断与治疗水平。方法回顾性分析4例非典型部位的DNT患者的临床表现，影像学检查，手术及病理结果。结果4例患者主要以头痛症状起病，癫痫发作较少，影像学表现特殊。免疫组化染色与常见的皮质DNT相似。所有患者进行手术治疗，随访1～6年，所有患者均可生活自理，头痛症状完全缓解，记忆力及行走不稳得到明显改善。结论非典型部位DNT临床表现较特殊，与肿瘤阻塞脑脊液循环通路有关，可与此部位发生的其他肿瘤相鉴别。术前充分评估肿瘤的自身特点，选择合适的手术入路，掌握良好的显微手术操作技巧可完全切除。一般认为预后良好。

Abstract： ObjectiveTo investigate the clinical characteristics of atypical site dysembryoplastic neuroepithelial tumor(DNT) and improve the level of diagnosis and treatment. MethodsThe clinical manifestations, imaging examination, surgical and pathological results of 4 patients with atypical DNT were analyzed retrospectively. Results The 4 patients mainly started with headache, had fewer seizures with special imaging manifestations. Immunohistochemical staining was similar to the common cortical DNT. All patients underwent surgery and were followed up for 1-6 years. All patients could take care of themselves, the symptoms of headache were completely relieved, and the memory and walking instability were significantly improved. ConclusionsThe clinical manifestation of atypical site DNT is special, which is related to the obstruction of cerebrospinal fluid circulation pathway by tumor, and can be distinguished from other tumors in this site. The tumor can be completely removed by fully evaluating its own characteristics, selecting an appropriate surgical approach and mastering good microsurgical skills. It is generally believed that the prognosis is good.