摘要
目的:探讨儿童头颈部神经源性肿瘤的临床特点、影像学特点及治疗方法,提高对该病的诊治经验。方法:对2015-01-2018-12期间收治的病理诊断为神经源性肿瘤的患儿病理类型、临床表现、影像学特点、治疗进行回顾性分析。结果:共29例患儿,神经鞘瘤5例,年龄9~14岁;神经纤维瘤5例,年龄9个月~9岁;神经母细胞源性肿瘤19例,年龄3个月~5岁8个月。头颈部神经源性肿瘤主要以颈部包块、睡眠打鼾为主要表现,神经母细胞源性肿瘤常伴有Honer综合征。神经源性肿瘤MRI通常表现为等长T1等长T2不均质信号。神经鞘瘤影像学特点为界清、有完整包膜,可见鼠尾样改变;神经纤维瘤影像学表现边界不清,丛状神经纤维瘤表现为多发团块状,可见分隔;神经母细胞性肿瘤超声具有低回声、不均质、点状钙化的特点。全部患儿进行了颈部肿物切除术,5例神经鞘瘤术中证实肿瘤来源于迷走神经,可完整剥除,术后无并发症出现;5例神经纤维瘤,2例术中肉眼下完整切除,2例仅做了部分切除术,1例位于食管入口者先后进行了3次支撑喉镜下肿物切除术;19例神经母细胞源性肿瘤中6例病变较局限,予以直接切除,13例予以化疗后手术切除,其中2例术后复发再次进行手术切除。结论:儿童头颈部神经源性肿瘤以神经母细胞瘤发病率最高,且发病年龄低,影像学上大多数神经源性肿瘤有其相应的特征表现,临床中应注意鉴别。神经鞘瘤边界清晰,包膜完整,较容易完整切除。头颈部神经纤维瘤因边界不清且常常病变广泛,周边紧邻血管、神经,难以保证安全界。神经母细胞瘤需根据病变范围、分期等决定治疗方案,大多数患儿由于肿瘤范围较广,包绕血管,术中考虑到保护神经等原因难以彻底切除,一般需结合术前化疗。
Objective:To investigate the clinical characteristics,imaging features and treatment of neurogenic tumor in chilehood and to improve the experience in diagnosis and treatment for the disease.Method:The twenty-nine inpatients of histopathologically proven neurogenic tumor from January 2015 to December 2018 were retrospectively analyzed.The pathological types,clinical characteristics,imaging findings,and management were analyzed.Result:There were five cases of schwannoma aged from 9 years to 14 years,five cases of neurofibroma aged from 9 months to 9 years,and nineteen cases of neuroblastic tumor aged from 3 months to 5 years in our series.The chief manifestations were soft tissue masses,snore,and Honer syndrome.As the tumors had different components pathologically,on scans they presented as masses with heterogeneous density.Schwannoma showed as oval masses with clear margins,with the characteristic of'tail sign'.The imaging findings of neurofibromas showed unclear boundaries and plexiform neurofibromas showed multiple clumps with visible separation.The ultrasonography of neuroblastic tumor was characterized by hypoechoic,heterogeneous and spotty calcification.All the cases underwent surgical resections.In 5 cases of schwannoma,it was confirmed that the tumors originated from the vagus nerve during the operation,which could be completely removed without postoperative complications.Two cases of neurofibromas were completely resected,2 cases were only partially resected,and 1 case which located at the entrance of the esophagus was resected under supportive laryngoscopic for three times.Thirteen of 19 children with neuroblastogenic tumors underwent resection in our department after chemotherapy,and 2 patients with postoperative recurrence underwent another operation.Conclusion:Neuroblastoma is the most common neurogenic tumor in the head and neck of children.Most neurogenic tumors have their corresponding characteristics on imaging and should be differentiated.Schwannoma has clear boundary and intact capsule,so it is easier to complete resection.The neurofibroma is commonly difficult to remove with safety margin because of its unclear boundaries.Neuroblastoma should be treated according to the lesion range and stage.Most of the lesions are difficult to remove completely due to the wide range,peripheral blood vessels and important nerves.So preoperative chemotherapy is generally required.
作者
李艳珍
刘雨薇
王生才
邰隽
张杰
刘原虎
李晓丹
张雪溪
葛文彤
倪鑫
LI Yanzhen;LIU Yuwei;WANG Shengcai;TAI Jun;ZHANG Jie;LIU Yuanhu;LI Xiaodan;ZHANG Xuexi;GE Wentong;NI Xin(National Children's Medical Center,Department of Otolaryngology Head and Neck Surgery,Beijing Children's Hospital,Capital Medical University,Beijing,100045,China)
出处
《临床耳鼻咽喉头颈外科杂志》
CAS
北大核心
2019年第10期983-986,共4页
Journal of Clinical Otorhinolaryngology Head And Neck Surgery
