Clinical characteristic and survival analysis of childhood supratentorial primitive neuroectoderma:a center re-trospective study
10.3760/cma.j.issn.2095-428X.2018.15.012
- VernacularTitle:儿童幕上原始性神经外胚瘤的临床特点和生存分析:单中心回顾性研究
- Author:
Siqi REN
1
;
Shuxu DU
;
Shuting LI
;
Jin ZHANG
;
Miao LI
;
Xiaojun GONG
;
Yanling SUN
;
Wanshui WU
;
Liming SUN
Author Information
1. 100038,首都医科大学附属北京世纪坛医院儿科
- Keywords:
Child;
Primitive neuroectoderma;
Central nervous system;
Chemotherapy
- From:
Chinese Journal of Applied Clinical Pediatrics
2018;33(15):1161-1164
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the survival rate and adverse effect of therapy of childhood supratentorial primitive neuroectoderma (sPNET)patients in order to investigate a suitable therapy. Methods Between December 2012 and December 2014,18 children,aged from 0. 6 to 13. 6 years old,were treated with pediatric himtumor(HIT)-2000 protocol in Beijing Shijitan Hospital,Capital Medical University. All patients were performed tumor gross total re-section,and someone accepted irradiation therapy firstly. The adverse effect of radiotherapy and chemotherapy was ana-lyzed,and the 3 - year progression - free survival (PFS)rate and overall survival (OS)rate were calculated by using Kaplan - Meier method. Results Till September 2017,the mean follow - up time was 3. 0 years (0. 2 - 4. 8 years), and the survival rate was 72. 2% (13 / 18 cases). Eleven cases(11 / 18 cases,61. 1%)were complete remission,but 7 cases (7 / 18 cases,38. 9%)relapsed during or after therapy. The 3 - year PFS and OS rates were (66. 7 ± 11. 1)%and (71. 8 ± 10. 7)%,respectively. Moreover,14 patients received craniospinal irradiation,and their 3 - year PFS and OS rates were (68. 8 ± 13. 3)% and (85. 7 ± 9. 4)%,respectively,there was no significant difference between the overall rates(all P >0. 05). During the follow - up period,all patients were not involved radiation - reduced brain necro-sis,myelitis,and pancreatic injury. During the whole central radiotherapy,14 children had different degree of blood rou-tine abnormalities,mainly manifested in leukocytes and thrombocytopenia,and the blood routine was restored to normal after subcutaneous injection of granulocyte stimulating factor. The adverse reactions of the gastrointestinal tract were re-latively mild,mainly nausea and vomiting,and the improvement of the gastric mucosa and the protection of the gastric mucosa were all improved. The gastrointestinal tract adverse reactions caused by HIT - 2000 were mainly nausea,vomi-ting,abdominal pain,diarrhea,constipation,and so on. The incidence of hematological adverse reactions was high,up to 100%,of which 2 cases were cured by active anti infection and symptomatic treatment due to the lack of granulocytic and fever. Conclusion HIT - 2000 protocol is a feasible and efficient therapy method for sPNET of Chinese children patients,and the adverse effects are tolerable.