摘要
目的探讨儿童晚期神经母细胞瘤（NB）合并颅内转移的临床特点、治疗方法及预后。方法回顾性分析北京同仁医院南区儿科病房2005年8月至2013年2月收治的14例晚期NB合并颅内转移患儿的临床资料。男8例，女6例；平均年龄5．5岁，均为Ⅳ期，对其肿瘤原发部位、转移部位、症状、治疗方式及生存时间进行分析。探讨自体外周血于细胞移植（APBSCT）治疗与颅内转移的关系。结果（1）临床症状主要为头痛、抽搐（各10例），恶心、呕吐（9例），头部多发包块、突眼（各6例）。（2）原发瘤来源于腹部肾上腺及盆腔交感神经链11例，来源于后纵隔2例，来源于鼻腔1例。颅内转移患儿中脑内肿块实质转移者11例，脑膜转移告4例，脑脊液可见瘤细胞者2例，合并骨髓转移9例，多发性骨转移12例，颅骨转移9例，眼眶转移6例。（3）8例患儿行APBSCT治疗，4例行病灶局部放射性治疗，3例行间碘苄胍（^131I-MIBG）治疗。颅内转移距APBSCT后，完伞缓解期3例患儿分别为22．0、12．0、4．3个月；部分缓解期2例患儿分别为41．0、36．8个月；进展期3例患儿分别为1．2、3、2．2个月。完全及部分缓解期与进展期行APBSCT治疗后发生颅内转移时间比较，差异有统计学意义（x^2=6．25，P=0．044）；失访1例。（4）随访至2013年2月，平均随访时间为（30．74±1967个月。NB合并颅内转移者死亡9例（64.3%），行APBSCT者发生颅内转移者死亡7例，死亡距颅内转移时间移植前缓解者平均为7.3个月，移植前进展者为1.1个月，差异有统计学意义（x^2=5.0,P=0.025）.NB患儿发病至颅内转移后死亡生存时间为移植前达完全或部分缓解者平均为43.4个月，移植前进展者为15.2个月，差异有统计学意义（x^2=5。0，P=0.025）；未行APBSCT者发生颅内转移者死亡2例，生存时间分别为60、38个月。余3例继续治疗，目前生存时间分别为14、42、13个月；1例失访。结论 儿童晚期NB合并颅内转移预后差，病死率高，APBSCT治疗宜于缓解期进行，可延迟发生颅内转移时间，临床需联合应用多种治疗方法提高缓解率，预防发生颅内转移。
Objectiv To explore the clinical features and outcome of advanced neuroblastoma（NB） with intracranial metastasis in children. Methods Retrospective review was performed on 14 cases（8 boys,6 girls） with ad- vanced NB who suffered from intracranial metastasis from Aug. 2005 to Feb. 2013 in Pediatrics of Beijing Tongren Hospital. The average age was 5i years old. Their primary sites of NB, metastatic sites, symptoms, therapy and survival time were analyzed, to investigate ate the relationship between the treatment of autologous peripheral blood stem cell trans- plantation（APBSCT） and intracranial metastasis. Results （ 1 ） In main clinical features of the children, 10 cases with headache, 10 cases with hyperspasmia,9 cases with nausea or vomiting,6 cases with many of the block in head ,6 eases with exophthalmos. （2）In the I majority of primary tumors, 11 cases arise in sympathetic nervous system of the abdomen and pelvic cavity, I 1 cases of these arising in posterior mediastinum, 1 case arising in cavum nasi. The intracranial metastasis expression of these children with N B were：the brain parenchymal metastasis tumor 11/14 eases, meningeal me- tastasis 4/14 cases, visible tumor cells in cerebrospinal fluid 2/14 cases,9 cases with bone marrow metastasis, 12 cases with multiple bone metastml 19 cases with multiple skull metastasis ,6 cases with the orbital transfer. （3） Eight cases were treated with APBSCT,including 3 eases of complete remission ,partial remission in 2 eases,3 cases of advanced ,4 cases of focal radiotherapy,3 eases of ^131 I-metaiodobenzylguanidine （^131 I-MIBG ） therapy. Intracranial metastasis from time of APBST, the 3 cases complete remission was 22.0 months, 12.0 months ,4.3 months, respectively ; the 2 cases of partial remission was 41.0 nonths ,36.8 months, respectively ; the 3 eases of advanced was 1.2,2.3,2.2 months, re- spectively. The occurrence dl ntracranial metastasis time was statistically significant（X2 = 6. 25, P = 0. 044） after APB- SCT in total and partial rein ion compared with the advanced cases. One case was lost in follow up. （4） The average follow-up time was（ 30.74 ± 67 ） months and until Feb. 2013. Nine eases died（64.3% ） who had NB with intracra- nial metastasis,while 7 casa gho took the APBSCT treatment died of intracranial metastasis,and the average time from intraeranial metastasis to dell was 7.3 mouths so it was statistically significant （X2 = 5.0 ,P = 0.025 ） in eases with re- mission NB before transplantatiion compared with 1.1 months in cases with advanced NB before transplantation;but the average time was not statistically significant （X2 = 5.4, P = 0.067 ） compared with the APBSCT patients. The survival time of NB children from tumor onset to death of intracranial metastasis was ： the average time was 43.4 months in cases with remission NB before transplantation compared with 15.2 months in cases with advanced NB before transplantation, which had statistical significance（x2 = 5.0, P = 0.025 ）. Two cases who hadn＇t APBSCT died of intracranial metastasis, in which the survival time was 60 months, 58 months, respectively. The remaining 3 cases continued to receive treat- ment,now the survival time was 14 months,42 months and 13 months. One case was lost in follow up. Conclusions The prognosis is poor in cases of children with advanced NB combined intracranial metastasis. APBSCT should be car- ried on during remission period ,as it could delay the occurrence of intracranial metastases. To prevent the occurrence of intracranial metastasis, various methods of treatment should be combined in NB.
出处
《中华实用儿科临床杂志》
CAS
CSCD
北大核心
2013年第15期1142-1145,共4页
Chinese Journal of Applied Clinical Pediatrics
基金
北京市科学技术员会首都临床特色应用研究项目（D101100050010052）
关键词
神经母细胞瘤
颅内转移
儿童
Neuroblastoma
Intracranial metastases
Child
