成人幕上多发原始神经外胚层肿瘤1例及文献复习

One case report of audlt supratentorial multiple primitive neuroectodermal tumor and literature review

目的探讨成人幕上原始神经外胚层肿瘤(PNET)的诊断及治疗,提高早期诊断率,改善疗效及预后。方法回顾性分析重庆市肿瘤医院2015年3月收治的1例成人幕上多发PNET患者。患者影像学检查示颅内多发占位病变,行颅内病灶切除术,术后根据病理检查结果行放疗。并以"Pub Med"及"中国知网"为检索工具复习相关文献。结果患者肿瘤大部分切除,术后病理检查为中枢神经系统原始外胚层肿瘤,行放疗。患者术后4个月时病情进展,术后9个月死亡。文献检索结果显示,国内外2005~2015年报道成人幕上PNET的文献共48篇。其中Pub Med搜索文献26篇,病例报告18篇,回顾性分析8篇;"中国知网"搜索文献22篇,病例报告14篇,回顾性分析8篇。共含病例276例,尚未见到成人幕上多发PNET病例的报道。结论 PNET发病率低,恶性程度极高,复发及转移率高;应早期诊断并及时手术治疗,辅以放、化疗综合治疗可延长患者的寿命。

Objective To explore the diagnosis and treatment of adult supratentorial primitive neuroectodermal tumor（ PNET）,to improve the early diagnostic rate,to improve the curative effect and prognosis. Methods Retrospective analysis of one audlt supratentorial multiple primitive neuroectodermal tumor who was in our hospital in March 2015,the patient imaging prompted intracranial multiple sites,and he did surgical operation treatment,most of the tumor resected,he accepted radiotheraphy according to postoperative pathology. And used ＂Pubmed＂and＂China net＂to review relevant literature. Result Most of the tumor resected,the patient＇s postoperative pathological prompted the central nervous system primitive neuroectodermal tumor,he accepted postoperative radiotherapy. By follow-up the condition of the patient worsed after postoperative four month and died after nine month. Retrieval results suggested： thereports of audlt supratentorial PNET from home and abroad,from 2005 to 2015,were 48 literature reports,including Pubmed searched 26 references,18 case reports of intracranial PNET,8 cases of retrospective analysis,＂CNKI ＂search with 22 references,14 case reports of intracranial PNET,8 cases retrospective analysis,included 276 cases,however there had none case of the audlt supratentorial multiple PNET been reported. Conclusion The incidence rate of adult supratentorial PNET was low,high malignant degree,high rate of recurrence or metastasis,should be early diagnosis and timely surgical treatment,it maybe prolong patient life by treatment with radiation and chemotherapy.