CAJ | 학술논문

目的提高对气管外周原始神经外胚层瘤(PNET)的认识.方法报道1例2010年7月确诊的气管外周PNET患者的临床资料、诊治经过及随访情况,并进行相关文献复习.以“原始神经外胚层瘤,外周”为检索词通过万方医学数据库对中文文献进行检索,以“primitive neuroeetodermal tumor,peripheral”为检索词通过PubMed数据库进行检索,检索时间截至2011年9月.结果患者女,63岁,因“咳嗽、咳痰50 d,气促1个月”入院.入院前曾误诊为“慢性咽炎、慢性支气管炎、支气管哮喘”.入院后胸部CT示气管中段占位性病变.支气管镜检查示声门下4 cm处可见带蒂肿物,肿物包膜完整,表面光滑,血管丰富,行支气管镜下气管肿物高频圈套电切电凝术.术后病理报告支气管黏膜下可见片状小圆形恶性肿瘤细胞浸润,免疫组织化学染色CD56及CD99呈弥漫强阳性,突触素及S-100蛋白阳性；白血病融合转录因子-1(FLI-1)融合基因表达阳性；确诊为气管外周PNET.患者术后咳嗽、气促症状消失,随访至今14个月,未见肿瘤局部复发及远处转移.在万方医学数据库上检索到40篇文献共187例PNET,24篇为回顾性分析,16篇为个案报道；在PubMed检索系统检索到111篇文献,1篇为前瞻性研究,1篇为综述,22篇为回顾性分析,87篇为个案报道,但均未见外周气管PNET的报道.结论气管外周PNET可发生于气管,易误诊,诊断主要依靠组织病理学及免疫组织化学染色,EWS-FLI-1融合基因阳性可作为确诊气管外周PNET的可靠指标；气管外周PNET可能与发生于其他部位的PNET不同,其侵袭性低,远处转移较少.
목적 제고대기관외주원시신경외배층류(PNET)적인식.방법 보도1례2010년7월학진적기관외주PNET환자적림상자료、진치경과급수방정황,병진행상관문헌복습.이“원시신경외배층류,외주”위검색사통과만방의학수거고대중문문헌진행검색,이“primitive neuroeetodermal tumor,peripheral”위검색사통과PubMed수거고진행검색,검색시간절지2011년9월.결과 환자녀,63세,인“해수、해담50 d,기촉1개월”입원.입원전증오진위“만성인염、만성지기관염、지기관효천”.입원후흉부CT시기관중단점위성병변.지기관경검사시성문하4 cm처가견대체종물,종물포막완정,표면광활,혈관봉부,행지기관경하기관종물고빈권투전절전응술.술후병리보고지기관점막하가견편상소원형악성종류세포침윤,면역조직화학염색CD56급CD99정미만강양성,돌촉소급S-100단백양성；백혈병융합전록인자-1(FLI-1)융합기인표체양성；학진위기관외주PNET.환자술후해수、기촉증상소실,수방지금14개월,미견종류국부복발급원처전이.재만방의학수거고상검색도40편문헌공187례PNET,24편위회고성분석,16편위개안보도；재PubMed검색계통검색도111편문헌,1편위전첨성연구,1편위종술,22편위회고성분석,87편위개안보도,단균미견외주기관PNET적보도.결론 기관외주PNET가발생우기관,역오진,진단주요의고조직병이학급면역조직화학염색,EWS-FLI-1융합기인양성가작위학진기관외주PNET적가고지표；기관외주PNET가능여발생우기타부위적PNET불동,기침습성저,원처전이교소.
Objective To improve the understanding of tracheal peripheral primitive neuroectodermal tumor(PNET).Methods A case of tracheal PNET diagnosed in July 2010 was reported and the related literatures were reviewed.The literature review was carried out respectively with “primitive neuroectodermal tumor”,“peripheral” as the search terms in Wanfang med online and PubMed database by September 2011.Results A case of 63 year-old female patient,who had been misdiagnosed as having chronic pharyngitis,chronic bronchitis and bronchial asthma,was admitted to the hospital because of cough and sputum production for 50 days,and anhelation for 1 month.After admission,the chest computerized tomography showed a space-occupying lesion in the middle of the trachea.Bronchoscopy showed a pedicle neoplasm 4 cm under the subglottic,with integral capsule,smooth surface and rich vascellum.Subsequently,tumor resection under bronchoscope was performed.Pathology report after operation showed infiltration of flake small round malignant cells under bronchial mucosa.Immunohistochemistry showed CD99 (+),Syn (+)and S-100 (+).EWS-FLI-1 fusion transcript was detected by RT-PCR.Accordingly,it was diagnosed as PNET.The symptoms of cough and anhelation were disappeared after operation.So far,there was no local recurrence and distant metastasis with 14 months follow-up.A total of 111 literatures were received in Pubmed,including one of prospective study,one of review,22 of retrospective study and 87 of case report.Forty literatures and 187 cases in all were received in Wanfang Med Online,including 24 of retrospective study and 16 of case report.But,there were no reports about tracheal PNET.Conclusions PNET can occur in the trachea and is easy to be misdiagnosed.To make a definite diagnosis,histopathology and immunohistochemistry are needed and detection of EWS-FLI-1 fusion transcript is a reliable marker for molecular diagnosis.The tracheal pPNET may be different with the pPNETs in other parts,and has a lowergrade invasion and less distant metastasis.