Abstract
Objective This study aimed to assess the development and current state
of management and outcome for neuroblastoma (NB) in Shanghai China.
Methods The clinical characteristics and survival rates of a large
cohort of 717 NB cases in the recent 10 years from two children’s
medical institutions in Shanghai China were retrospectively analyzed.
Results The 8y-EFS and OS of the whole cohort in the 10 years were
67.6±2.2% and 81.2±2.1%. Our risk stratification system was updated
twice during the 10 years, forming three periods. The percentage of very
low risk (VLR) cohort who accepting only surgery without chemotherapy
was increased in 2016-2018 period than the 2010-2015 period and
2008-2009 period. While the 3y-EFS of the three periods were similar
(P=0.961). The outcome in VLR and low risk (LR) patients were excellent
with 8y-EFS around 92% (VLR=93.0±2.8%, LR= 92.1±1.8%). The outcome in
high risk (HR) patients was significantly poorer with 8y-EFS only as
16.6±4.1% even than intermediate risk (IR) patients with 8y-EFS as
69.6±4.4% (P<0.001). Conclusions The revision of our risk
stratification system was effective, making an increasing percentage of
patients without chemotherapy while with similar EFS rates. The VLR and
LR cohort had excellent outcomes, however the HR cohort with most of the
mortality remains one of the greatest challenges. Enriching the
transplant resources, importing melphalan to make ASCT more available
and effective, and importing advanced novel therapies like anti-GD2
antibody and 131I-mIBG are our objectives to improve the survival of the
HR patients.