Background: “Rare cases of Burkitt leukemia/lymphoma (BL) exhibiting a precursor B-cell phenotype (termed herein pre-BLL) were admitted by WHO Classification of Hematopoetic and Lymphoid Tissue, recent evidence suggests that these neoplasms genetically and epigenetically resemble precursor B-cell leukemia/lymphoma (pB-acute lymphoblastic leukemia/lymphoblastic lymphoma (ALL)/LBL) rather than BL. The clinical features and treatment of childhood pre-BLL with C-myC rearrangement are poorly understood. Methods：The clinical features, treatment strategies and follow-up information of 9 cases with pre-BLL diagnosed by Shanghai Children’s Medical Center affiliated to Shanghai Jiao Tong University School of Medicine from 2011 to 2020 were retrospectively analyzed. Results: All the 9 cases were confirmed to be pre-BLL by flow cytometry and fluorescence in situ hybridization, morphological classification were L2/L3, immunophenotype was CD10, CD19 positive, CD20, TDT, CD34 selective expression, sIgM negative, Kappa and Lambda light chain negative. Most of the pre-BLL cases were accompanied by elevated lactate dehydrogenase (LDH), uric acid levels. 5 cases received intensive chemotherapy with overlapping regimen, and all achieved sustained remission except for 1 case relapse and death. Among the 4 cases who received low-intensity chemotherapy for acute lymphoblastic leukemia, 2 cases died due to early relapse of the disease. Conclusions: Pre-BLL cases are rare. and intensive chemotherapy treatment according to protocols for mature B-cell NHL. Currently, the treatment strategies are still controversial. Considering the small number of cases, multi-center clinical studies should be actively carried out to find a standard treatment plan.

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.