Clinical characteristics and prognosis of children with nearly diploid neuroblastoma / 中华实用儿科临床杂志

ABSTRACT

Objective:To investigate the clinical characteristics, treatment effect and prognosis of children with nearly diploid neuroblastoma (NB).Methods:A retrospective analysis of the general clinical characteristics (including age, Gender, risk grouping, location of primary tumor, etc.), laboratory test results, treatment and recent prognosis of NB children with nearly diploidy in bone marrow chromosomes by G-banding technology who admitted to Beijing Children′s Hospital, Capital Medical University from January 2015 to December 2018. Kaplan- Meier method was adopted to calculate survival rate.Univariate analysis was performed using Log- Rank test, and multivariate analysis was conducted with Cox regression model. Results:A total of 43 patients, including 27 males and 16 females, with diagnosis were included, with 14 cases in the hypodiploid group and 29 cases in the hyperdiploid group, and the median age was 35.5 months.The 43 children were all in the high-risk group of International Neuroblastoma Staging System(INSS)-Ⅳ.The primary tumors were mainly located in the retroperitoneal adrenal region (83.7%, 36/43 cases). The largest diameter of the tumors was more than 10 cm (53.5%, 23/43 cases), and often accompanied by 2 or more metastases at the time of consultation.In terms of chromosome karyotype and chromosome karyotype of 14 children in the hypodiploid group was 41-45, the most common karyotype was 45 chromosomes[9 cases(64.3%)]. Among 29 children in the hyperdiploid group of the 47 chromosome karyotypes, 11 cases were common (37.9%). Tumor markers were as follows neuron enolase (NSE) increased in 41 cases children (95.3%) at first diagnosis, and 25 cases (58.1%)> 370 μg/L; 42 cases (97.7%)had lactate dehydrogenase (LDH). The LDH of children in the hypodiploid group was all> 500 U/L, with 1 case was> 10 000 U/L.Nine cases (20.9%) of MYCN gene were detected by fluorescence in situ hybridization (FISH). Treatment and prognosis the total course of chemotherapy for 43 patients was 1-12, 19(44.2%) patients received autologous stem cell transplantation, 21 patients (46.5%) received postoperative or autologous radiotherapy or metaiodobenzylguanidine treatment, 28 children developed or relapsed with a median duration of 13.8 months, and 15 cases (34.9%) died.The median follow-up time of the 14 children in the hypodiploid group was 14.9 months (2-38 months), 12 cases progressed or relapsed, and 7 died.The median follow-up of 29 children in the hyperdiploid group was 20.0 months (8.1-51.6 months), with 16 patients progressed or relapsed and 8 cases died. Kaplan- Meier survival analysis illustrated that the 3-year projected event free survival (EFS) rate of 43 children was 18.4%, of which 17.1% were in the hypodiploid group and 29.8% in the hyperdiploid group. Conclusions:Preliminary analysis reveals that children with nearly diploid NB are mostly in the stage Ⅳ high-risk group over the age of 18 months, and 2 or more metastases at the time of consultation.The 3-year estimated EFS of 43 children was 18.4%, and the prognosis was worse in the hypodiploid group.