< 0.01）；复发患儿平均生存时间（51.68个月）低于无复发病例（62.57个月），差异有统计学意义（P < 0.01）。结论 年长儿NB临床分期多属晚期，但规律治疗仍可提高疗效，应增加患儿信心，坚持规范治疗。"/>
Clinical features and outcomes of neuroblastoma patients aged above 5 years
Abstract Objective To investigate the clinical features and outcomes of neuroblastoma (NB) children aged above 5 years, and to provide a theoretical basis for improving prognosis. Methods A retrospective analysis was performed for the clinical data of 54 previously untreated NB children, and their clinical features and outcome were analyzed. The Kaplan-Meier method was used for survival analysis. Results Among the 54 children, there were 36 boys and 18 girls, and all of them had stage 3 or 4 NB. Of all the children, 41 (41/54, 76%) had retroperitoneal space-occupying lesions, 10 (10/54, 18%) had mediastinal space-occupying lesions, 2 (2/54, 4%) had intraspinal space-occupying lesions, and 1 (1/54, 2%) had pelvic space-occupying lesions. At the end of the follow-up, 30 children (30/54, 56%) survived, among whom 23 (77%) achieved disease-free survival (9 achieved complete remission after chemotherapy for recurrence), 6 (20%) achieved partial remission of tumor (all of them received chemotherapy again due to recurrence), and 1 (3%) experienced progression (with progression after chemotherapy again due to recurrence); 24 children (44%) died, among whom 22 died after chemotherapy again due to recurrence and 2 died of multiple organ failure during the first treatment. According to the Kaplan-Meier survival analysis, the mean survival time was 53.8 months, and the children with stage 3 NB had a significantly higher overall survival rate than those with stage 4 NB (80% vs 53%;P < 0.01). The children with recurrence had a significantly lower mean survival time than those without recurrence (51.68 months vs 62.57 months;P < 0.01). Conclusions Older children often have late-stage NB, but standard treatment can improve their outcomes.
Evans AE. Neuroblastoma[M]//Brodeur GM, Sawada T, Tsuchida Y, et al. A historical perspective 1984-1998. 2nd edition. American:Elsevier & Baylor University Press, 2000:1-19.
[2]
Akramipour R, Zargooshi J, Rahimi Z. Infant with concomitant presence of Hernia/Hydrocele and Primary Paratesticular Neuroblastoma.A diagnostic and therapeutic challenge[J]. J Pediatr Hematol Oncol, 2009, 31(5):349.
[3]
Moreno F, Lopez MJ, Palladino M, et al. Childhood neuroblastoma:incidence and survival in Argentina. Report from the National Pediatric Cancer Registry, ROHA network 2000-2012[J]. Pediatr Blood Cancer, 2016, 63(8):1362-1367.
[4]
Peinemann F, van Dalen EC, Berthold F. Rapid COJEC induction therapy for high-risk neuroblastoma patients-cochrane review[J]. Klin Padiatr, 2016, 228(3):130-134.
[5]
Hoy SM. Dinutrximab:a review in high-risk neuroblastoma[J]. Target Oncol, 2016, 11(2):247-253.
Ozerov SS, Samarin AE, Andreev ES, et al. Neurosurgical aspects of the treatment of neuroblastoma patients[J]. Zh Vopr Neirokhir Im N N Burdenko,2016, 80(3):50-57.
[8]
Escobar MA, Grosfeld JL, Powell RL, et al. Long-term outcomes in patients with stage IV neuroblastoma[J]. J Pediatr Surg, 2006, 41(2):377-381.
[9]
Tapia FJ, Polak JM, Barbosa AJ, et al. Neuron-specific enolase is produced by neuroeudocrine tumours[J]. Lancet, 1981, 1(8224):808-811.
[10]
Carter DR, Sutton SK, Pajic M, et al. Glutathione biosynthesis is upregulated at the initiation of MYCN-driven neuroblastoma tumorigenesis[J]. Mol Oncol, 2016, 10(6):866-878.
Rojas Y, Jaramillo S, Lyons K, et al. The optimal timing of surgical resection in high-risk neuroblastoma[J]. J Pediatr Surg, 2016, S0022-3468(16):30110-30115.
