神经纤维瘤病Ⅰ型合并儿童恶性实体肿瘤的临床分析  

Clinical analysis of neurofibromatosis type I complicated with malignant solid tumor in children

作　　者：贺思豆 苏雁[1] 段超[1] 张大伟[1] 金眉[1] 于彤[2] 伏利兵[3] 马晓莉[1] HE Sidou;SU Yan;DUAN Chao;ZHANG Dawei;JIN Mei;YU Tong;FU Libing;MA Xiaoli(Department of Cancer Center,Department of Oncology and Beijing Children′s Blood Tumor Key Laboratory,Key Laboratory of Pediatric Major Diseases Research,Ministry of Education,National Children′s Medical Center,Beijing Children′s Hospital,Capital Medical University;Department of Imaging,National Children′s Medical Center,Beijing Children′s Hospital,Capital Medical University;Department of Pathology,National Children′s Medical Center,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院,儿童肿瘤中心肿瘤内科,北京市儿童血液肿瘤重点实验室,儿科重大疾病研究教育部重点实验室 [2]国家儿童医学中心,首都医科大学附属北京儿童医院影像中心,北京100045 [3]国家儿童医学中心,首都医科大学附属北京儿童医院病理科,北京100045

出　　处：《中国小儿血液与肿瘤杂志》2023年第2期93-96,102,共5页Journal of China Pediatric Blood and Cancer

基　　金：北京市研究型病房项目(BCR202101);北京市自然科学基金(7222054)

摘　　要：目的 总结神经纤维瘤病Ⅰ型(NF1)合并恶性实体肿瘤患儿的临床特征及预后。方法 回顾性分析2018年-2022年在我院肿瘤内科诊断并治疗随访的NF1合并恶性实体肿瘤患儿的临床资料,并分析预后。结果 共8名患儿纳入分析,男女各4例。诊断NF1的中位年龄为6.8(0.9-12.9)岁,诊断恶性实体肿瘤的中位年龄为5.3(0.9-13.3)岁。6例在实体肿瘤诊断时或之后确诊为NF1,仅2例在肿瘤诊断前确诊。肿瘤包括3例恶性周围神经鞘瘤、2例横纹肌肉瘤、2例视神路胶质瘤和1例神经母细胞瘤。所有患儿接受化疗;7例进行手术治疗,其中6例完全切除;4例接受放疗。随访中位时间为12.5(1-68)个月,7例存活,复发3例。结论 NF1可合并多种恶性实体肿瘤,早期识别NF1合并恶性肿瘤。Objective To summarize the clinical features and prognosis of children with neurofibromatosis type I(NF1)complicated with malignant solid tumor.Methods The clinical data of NF1 patients with malignant solid tumors diagnosed in the department of oncology in our hospital from^(2)018 to 2022 were retrospectively studied.Results A total of 8 children were recruited in this study,including 4 males and 4 females.The median age at diagnosis of NF1 was 6.8(0.9 to 12.9)years.The median age of the 8 children diagnosed with malignant solid tumors was 5.3(0.9-13.3)years.Six patients were diagnosed with NF1 at the same time or after tumor diagnosis,only 2 were diagnosed before tumor diagnosis.Malignant solid tumors included 3 malignant peripheral schmomas,2 rhabdomyosarcomas,2 optic gliomas,and 1 neuroblastoma.8 cases received chemotherapy for malignant solid tumors,7 cases received surgical treatment,of which 6 cases received complete resection and 4 cases received radiotherapy.The median follow-up time was 12.5(1-68)months.Seven patients survived and 3 patients had tumor recurrence.Conclusions NF1 can combine with multiple malignant solid tumors.For children with NF1 combined with malignant tumors should be early recognited.

关 键 词：儿童 NF1 恶性实体肿瘤 临床特征 

分 类 号：R739.4[医药卫生—肿瘤]