title: Multivariate analysis of risk factors for patients with stage 4 neuroblastoma who were older than 18 months at diagnosis: a report from a single institute in Shanghai, China.
abstract: This retrospective study evaluated the long-term outcomes and prognostic indicators of patients with stage 4 neuroblastoma who were older than 18 months at diagnosis. The medical records of 118 such children who were treated at Shanghai Children's Medical Center, China, from June 1998-December 2013 were reviewed. Event-free survival (EFS) and overall survival (OS) were analyzed by log-rank tests. Of the 118 patients, 14 improving patients did not complete treatment because of parental decisions, and 1 patient died during surgery. Of the 103 patients who completed the comprehensive protocols, 60 (58.3%) achieved very good partial remission (VGPR), 26 (25.2%) achieved partial remission (PR) after four courses of chemotherapy, and 17 (16.5%) progressed during treatment. The response to induction (including VGPR + PR) was 83.5%. After a median follow-up of 105 months (range 36-160 months), the 5- and 10-year OS were 21 and 18%, and the EFS was 19 and 13%, respectively. EFS was significantly better for patients with normal levels of urinary vanillylmandelic acid (VMA) at diagnosis, who had complete resection of the primary tumor, who were minimal residual disease- (MRD-) negative in their bone marrow after four courses of chemotherapy, and who achieved VGPR at the end of treatment (P < 0.05). The prognosis remains poor for patients with stage 4 neuroblastoma who are older than 18 months at diagnosis. Elevated VMA level, incomplete tumor resection, persistent MRD in bone marrow, and poor curative effect are associated with worse prognosis.
authors.affiliation: Key Laboratory of Pediatric Hematology and Oncology, Ministry of Health, Department of Pediatric Hematology and Oncology, Shanghai Children's Medical Center, Shanghai JiaoTong University School of Medicine (SJTU-SM), Dongfang Road 1678, Shanghai, 200127, China.
authors.forename: Jiaoyang
authors.initials: J
authors.lastname: Cai
authors.affiliation: Key Laboratory of Pediatric Hematology and Oncology, Ministry of Health, Department of Pediatric Hematology and Oncology, Shanghai Children's Medical Center, Shanghai JiaoTong University School of Medicine (SJTU-SM), Dongfang Road 1678, Shanghai, 200127, China.
authors.forename: Ci
authors.initials: C
authors.lastname: Pan
authors.affiliation: Key Laboratory of Pediatric Hematology and Oncology, Ministry of Health, Department of Pediatric Hematology and Oncology, Shanghai Children's Medical Center, Shanghai JiaoTong University School of Medicine (SJTU-SM), Dongfang Road 1678, Shanghai, 200127, China.
authors.forename: Yanjing
authors.initials: Y
authors.lastname: Tang
authors.affiliation: Key Laboratory of Pediatric Hematology and Oncology, Ministry of Health, Department of Pediatric Hematology and Oncology, Shanghai Children's Medical Center, Shanghai JiaoTong University School of Medicine (SJTU-SM), Dongfang Road 1678, Shanghai, 200127, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Chen
authors.affiliation: Key Laboratory of Pediatric Hematology and Oncology, Ministry of Health, Department of Pediatric Hematology and Oncology, Shanghai Children's Medical Center, Shanghai JiaoTong University School of Medicine (SJTU-SM), Dongfang Road 1678, Shanghai, 200127, China.
authors.forename: Min
authors.initials: M
authors.lastname: Zhou
authors.affiliation: Key Laboratory of Pediatric Hematology and Oncology, Ministry of Health, Department of Pediatric Hematology and Oncology, Shanghai Children's Medical Center, Shanghai JiaoTong University School of Medicine (SJTU-SM), Dongfang Road 1678, Shanghai, 200127, China.
authors.forename: Benshang
authors.initials: B
authors.lastname: Li
authors.affiliation: Key Laboratory of Pediatric Hematology and Oncology, Ministry of Health, Department of Pediatric Hematology and Oncology, Shanghai Children's Medical Center, Shanghai JiaoTong University School of Medicine (SJTU-SM), Dongfang Road 1678, Shanghai, 200127, China.
authors.forename: Huiliang
authors.initials: H
authors.lastname: Xue
authors.affiliation: Key Laboratory of Pediatric Hematology and Oncology, Ministry of Health, Department of Pediatric Hematology and Oncology, Shanghai Children's Medical Center, Shanghai JiaoTong University School of Medicine (SJTU-SM), Dongfang Road 1678, Shanghai, 200127, China.
authors.forename: Shuhong
authors.initials: S
authors.lastname: Shen
authors.affiliation: Key Laboratory of Pediatric Hematology and Oncology, Ministry of Health, Department of Pediatric Hematology and Oncology, Shanghai Children's Medical Center, Shanghai JiaoTong University School of Medicine (SJTU-SM), Dongfang Road 1678, Shanghai, 200127, China.
authors.forename: Yijin
authors.initials: Y
authors.lastname: Gao
authors.affiliation: Key Laboratory of Pediatric Hematology and Oncology, Ministry of Health, Department of Pediatric Hematology and Oncology, Shanghai Children's Medical Center, Shanghai JiaoTong University School of Medicine (SJTU-SM), Dongfang Road 1678, Shanghai, 200127, China.
authors.forename: AnAn
authors.initials: A
authors.lastname: Zhang
authors.affiliation: Key Laboratory of Pediatric Hematology and Oncology, Ministry of Health, Department of Pediatric Hematology and Oncology, Shanghai Children's Medical Center, Shanghai JiaoTong University School of Medicine (SJTU-SM), Dongfang Road 1678, Shanghai, 200127, China. tangjingyan@scmc.com.cn.
authors.forename: Jingyan
authors.initials: J
authors.lastname: Tang
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D002681:China; D018572:Disease-Free Survival; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D015999:Multivariate Analysis; D018365:Neoplasm, Residual / Q000401:mortality / Q000473:pathology; D009447:Neuroblastoma / Q000401:mortality* / Q000473:pathology* / Q000628:therapy*; D036102:Peripheral Blood Stem Cell Transplantation; D011379:Prognosis; D016016:Proportional Hazards Models; D012074:Remission Induction; D012189:Retrospective Studies; D012307:Risk Factors; D014642:Vanilmandelic Acid / Q000652:urine
chemical_list: D014642:Vanilmandelic Acid
keywords: Neuroblastoma; Risk factors; Stage 4