CAJ | 학술논문

目的探讨伴眼阵挛-肌阵挛综合征(OMS)的神经母细胞瘤(NB)患儿的综合治疗方法.方法 2011年5月至2013年12月,在北京大学第一医院儿科收治6例OMS-NB患儿,其中男4例、女2例,起病年龄13 ～ 24个月(中位年龄19.5个月),误诊2～ 14个月(中位7.5个月).确诊后给予术前化疗、手术和术后化疗等综合治疗,并进行了长期随诊.结合上述临床过程主要就肿瘤部位、分期、病理类型、治疗方式及预后进行了回顾性分析.结果所有患儿病初均误诊为单纯OMS,分别给予1～13个月的糖皮质激素和丙种球蛋白的治疗,均在疗效不佳或病情反复后才给予腹部增强CT检查,确诊为OMS-NB.肿瘤位于肾上腺、腹膜后或盆腔,分期Ⅰ或Ⅱ期,病理类型为节细胞神经母细胞型5例,为神经母细胞瘤1例.所有患儿明确诊断后都给予了以手术为主的综合治疗,其中4例手术完整切除肿瘤,2例因肿瘤包绕主动脉而切开包膜致局部有残留.2例患儿给予了术前化疗,5例患儿接受了术后化疗.6例患儿随访3 ～31个月,1例失访,余5例至2013年12月均无病存活(3例已结束化疗,1例局部复发后再次接受手术及术后放、化疗,现也已停药,1例仍在化疗中),其神经系统表现在术后化疗后都得到明显改善.结论所有临床诊断OMS患儿都应定期接受腹部和盆腔的影像学监测,以减少OMS-NB的延误诊断;对OMS-NB患儿应积极给予包括手术、化疗或放疗在内的综合治疗,以减少局部复发,并改善神经系统的长期预后,但化疗时间仍需进一步探讨.
목적 탐토반안진련-기진련종합정(OMS)적신경모세포류(NB)환인적종합치료방법.방법 2011년5월지2013년12월,재북경대학제일의원인과수치6례OMS-NB환인,기중남4례、녀2례,기병년령13 ～ 24개월(중위년령19.5개월),오진2～ 14개월(중위7.5개월).학진후급여술전화료、수술화술후화료등종합치료,병진행료장기수진.결합상술림상과정주요취종류부위、분기、병리류형、치료방식급예후진행료회고성분석.결과 소유환인병초균오진위단순OMS,분별급여1～13개월적당피질격소화병충구단백적치료,균재료효불가혹병정반복후재급여복부증강CT검사,학진위OMS-NB.종류위우신상선、복막후혹분강,분기Ⅰ혹Ⅱ기,병리류형위절세포신경모세포형5례,위신경모세포류1례.소유환인명학진단후도급여료이수술위주적종합치료,기중4례수술완정절제종류,2례인종류포요주동맥이절개포막치국부유잔류.2례환인급여료술전화료,5례환인접수료술후화료.6례환인수방3 ～31개월,1례실방,여5례지2013년12월균무병존활(3례이결속화료,1례국부복발후재차접수수술급술후방、화료,현야이정약,1례잉재화료중),기신경계통표현재술후화료후도득도명현개선.결론 소유림상진단OMS환인도응정기접수복부화분강적영상학감측,이감소OMS-NB적연오진단;대OMS-NB환인응적겁급여포괄수술、화료혹방료재내적종합치료,이감소국부복발,병개선신경계통적장기예후,단화료시간잉수진일보탐토.
Objective To investigate the efficacy of combined modality therapy for neuroblastoma in children associated with opsoclonus-myoclonus syndrome (OMS-NB).Method From May 2011 to December 2013,6 consecutive patients (4 boys and 2 girls) diagnosed as OMS-NB underwent surgery and chemotherapy in the First Hospital,Peking University.The median age of onset was 19.5 months (range 13-24 months) and misdiagnosis occurred 7.5 months (range 2-14 months) ago.A retrospective analysis for the location,stage,pathological type,treatment way and outcome of neuroblastoma was done.Result (1)All patients were misdiagnosed as simply opsoclonus-myoclonus syndrome (OMS) at the time of onset.They had been receiving treatment with adrenocorticotropic hormone and intravenous immunoglobulin within 1-13 months.OMS-NB was diagnosed by means of enhanced abdominal CT image which was delayed to be given after the poor efficacy or relapse.(2) The primary tumors were almost all small,stage Ⅰ-Ⅱ,located in adrenal,retroperitoneal or pelvis.The pathology of tumors included ganglioneuroblastoma (5/6) and neuroblastoma (1/6).(3) All these cases underwent surgery,4/6 cases with complete tumor resection,2/6 cases with tumor around the aorta and induced local residue.Preoperative and postoperative chemotherapy was given to 2 and 5 cases,respectively.(4) The patients were followed up for 3-31 months,except 1 patient lost,the other 5 are currently surviving disease-free (3 having been at the end of chemotherapy,1 still in chemotherapy,and another had local recurrence and is receiving radiotherapy and chemotherapy after the second operation and now also stopped taking the medicine).The symptoms of nervous system have been significantly improved during postoperative chemotherapy.Conclusion To reduce the misdiagnosis,regular CT imaging of the abdomen or pelvic should be ordered for all cases with OMS.The children with OMS-NB need to be actively treated with the combined modality therapy including surgery,chemotherapy or radiotherapy,to reduce recurrence and reduce the symptoms of nervous system.