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| 1 | Treatment and outcomes of 1041 pediatric patients with neuroblastoma who received multidisciplinary care in China显示文摘Importance:Neuroblastoma is the most common extracranial malignant solid tumor in children.Multidisciplinary care is critical to improving the survival of pediatric patients with neuroblastoma.Objective:To systematically summarize the clinical characteristics of children with neuroblastoma and evaluate their prognosis with multidisciplinary care provided in a single center.Methods:This retrospective study analyzed the clinical data of 1041 patients with neuroblastoma who were diagnosed,treated,and followed-up in the Hematology-Oncology Center of Beijing Children’s Hospital from 2007 to 2019.Results:The median age at diagnosis was 34 months;80.8%of the patients were younger than 5 years of age.Notably,243 patients(23.3%)were classified as low-risk,249 patients(23.9%)were classified as intermediate-risk,and 549(52.7%)were classified as high-risk.Furthermore,956 patients underwent surgical resections;986(94.7%)patients received chemotherapy;and 176 patients with high-risk neuroblastoma received hematopoietic stem cell transplantation.The 5-year event-free survival(EFS)rate was 91.3%and 5-year overall survival(OS)rate was 97.5%in low-risk group;in the intermediate-risk group,these rates were 85.1%and 96.7%,respectively,while they were 37.7%and 48.9%in the high-risk group(P<0.001 for both).The 5-year EFS and OS rates were significantly higher in patients diagnosed between 2015 and 2019 than in patients diagnosed between 2007 and 2014(P<0.001).In total,278 patients(26.7%)exhibited tumor relapse or progression;the median interval until relapse or progression was 14 months.Of the 233 patients who died,83%died of relapse or progression of neuroblastoma and 4.3%died of therapy-related complications.Interpretation:The 5-year OS rate was low in high-risk patients,compared with low-and intermediate-risk patients.Multidisciplinary care is critical for improvement of survival in pediatric patients with neuroblastoma.Additional treatment strategies should be sought to improve the prognosis of patients with high-risk neuroblastoma. | Yan Su Hong Qin Chenghao Chen Shengcai Wang Shihan Zhang Dawei Zhang Mei Jin Yun Peng Lejian He Xiaoman Wang Suyun Qian Maoquan Qin Ming Ge Fuquan Zhang Qi Zeng Huanmin Wang Xiaoli Ma Xin Ni | 2020 | Pediatric Investigation2020，4，3： | 16 |
| 2 | Correlation between BRAF^(V600E) mutation and clinicopathological features in pediatric papillary thyroid carcinoma显示文摘In adults, the presence of the BRAF^(V600E) mutation in papillary thyroid cancer(PTC) has been demonstrated to be strongly associated with aggressive cancer-cell characteristics and poor patient prognosis. In contrast, the frequency of this mutation in pediatric PTC has undergone limited study, and the few available estimates range from 0 to 63%. Furthermore, the role of the BRAF^(V600E) mutation in pediatric PTC is controversial; thus, the present study aimed to investigate the prevalence and role of the BRAF^(V600E) mutation in48 pediatric patients with PTC, aged 3–13 years. Of these patients, 41 were diagnosed with classic PTC, five were found to have a follicular variant of PTC, and two to exhibit a diffuse sclerosing PTC variant. The BRAF^(V600E) mutation was identified to be present in 35.4% of the 48 analyzed patients, and in 41.5% of the patients diagnosed with classical PTC. Furthermore, the presence of the BRAF^(V600E) mutation was found to be associated with a patient age at diagnosis of less than ten years(P=0.011), the performance of a thyroidectomy(P=0.03), exhibited tumor multifocality(P=0.02) and/or extra-thyroidal invasion(P=0.003), and both a low MACIS(Metastases, Age, Completeness of resection, Invasion, Size)(P=0.036) and AMES(Age, Metastasis, Extent of tumor,Size)(P=0.001)score. Together, these data suggest that the presence of the BRAF^(V600E) mutation may be negatively correlated with partial aggressive clinicopathological features of pediatric PTC. | Jiangqiao Geng Huanmin Wang Yuanhu Liu Jun Tai Yaqiong Jin Jie Zhang Lejian He Libing Fu Hong Qin Yingluan Song Jinzhu Su Aiying Zhang Xin Wen Yongli Guo Xin Ni | 2017 | Science China(Life Sciences)2017，60，7： | 9 |
| 3 | Acute myeloid leukemia following a primary mediastinal germ cell tumor显示文摘Introduction:There is a known association between primary mediastinal germ cell tumor(PMGCT)and hematologic malignancy that is not linked to treatment.They are exceptionally rare entities with a low morbidity and a poor prognosis.Case presentation:An 11-year-old boy presented with an anterior mediastinal mass diagnosed as a malignant germ cell tumor on the basis of an excisional biopsy.He was found to have acute myeloid leukemia(AML)two years after the chemotherapy for his germ cell tumor.The clinical course was very aggressive with a survival time of only 1 week after diagnosis of AML associated with PMGCT.Conclusion:AML associated with PMGCT needs to be diagnosed correctly.Relevant examinations should be carried out in patients with PMGCTs during and after chemotherapy,and long-term follow-up is still necessary to reduce the risk of morbidity and mortality. | Huimin Hong Yan Su Chenghao Chen Lejian He Shuai Zhu Wei Lin Mei Jin Xisi Wang Ruidong Zhang Huyong Zheng Qi Zeng Xiaoli Ma | 2020 | Pediatric Investigation2020，4，3： | 1 |
| 4 | DCX and CRABP2 are candidate genes for differential diagnosis between pre-chemotherapy embryonic and alveolar rhabdomyosarcoma in pediatric patients显示文摘Importance Rhabdomyosarcoma(RMS)is the most common soft tissue sarcoma in children.More than 90%of cases are classified as embryonic RMS(ERMS)or alveolar RMS(ARMS).ERMS has a worse prognosis than ARMS.Early differential diagnosis is of paramount importance for optimization of treatment.Objective To identify genes that are differentially expressed between ARMS and ERMS,which can be used for accurate rhabdomyosarcoma classification.Methods Three Gene Expression Omnibus datasets composed of ARMS and ERMS samples were screened and 35 differentially expressed genes(DEGs)were identified.Receiver operating characteristic curve analysis and area under the curve analysis was performed for these 35 DEGs and seven candidate genes with the best differential expression scores between ARMS and ERMS were determined.The expression of these seven candidate genes was validated by immunohistochemical analysis of pre-chemotherapy ARMS and ERMS specimens.Results The levels of DCX and CRABP2 were confirmed to be remarkably different between paraffin-embedded ARMS and ERMS tissues,while EGFR abundance was only marginally different between these two RMS subtypes.Interpretation DCX and CRABP2 are potential biomarkers for distinguishing ARMS from ERMS in pre-chemotherapy pediatric patients. | Nian Sun Yeran Yang Shengcai Wang Jie Zhang Jingang Gui Jun Tai Lejian He Jiatong Xu Yanzhen Li Xuexi Zhang Qiaoyin Liu Zhiyong Liu Yongli Guo Xin Ni | 2021 | Pediatric Investigation2021，5，2： | 0 |
| 5 | Primary cardiac CIC-rearranged undifferentiated sarcoma in an infant显示文摘Introduction:Cardiac neoplasms are particularly rare in children,and the majority of these tumors are benign.Approximately 10%of cardiac neoplasms are malignant,including soft tissue sarcomas and lymphomas.Cardiac tumors could also be metastases.Primitive EWSR1-negative round or spindle cell undifferentiated sarcoma harboring CIC gene translocation is a highly aggressive malignancy mainly occurring in soft tissues.However,it has not yet been described in the heart.Case presentation:We report a sarcoma that arose from the right ventricle in a 1-year-old girl.Histologically,it was composed of closely arranged small round or oval undifferentiated cells with fibrovascular separation,hyaline degeneration,and geographical necrosis.Immunohistochemically,the neoplastic cells exhibited focal membrane positivity for CD99 and diffuse positivity for WT1 and ETV4.Fluorescent in situ hybridization analysis showed EWSR1-negative but CIC-positive split signals.The breakpoint was also confirmed by whole genome sequencing.Conclusion:Based on morphological,immunohistochemical and molecular findings,this cardiac mass was diagnosed as CIC-rearranged sarcoma. | Meng Zhang Yeran Yang Xiaoxing Guan Xingfeng Yao Yongli Guo Lejian He | 2021 | Pediatric Investigation2021，5，4： | 0 |
| 6 | Detection of FOXO1 break-apart status by fluorescence in situ hybridization in atypical alveolar rhabdomyosarcoma显示文摘The morphologies of alveolar rhabdomyosarcoma(ARMS) are various. Some cases entirely lack an alveolar pattern and instead display a histological pattern that overlaps with embryonal rhabdomyosarcoma(ERMS). The method of pathological diagnosis of ARMS and ERMS has been updated in the 4th edition of the World Health Organization's guidelines for classification of skeletal muscle tumors. Under the new guidelines, there is still no molecular test to distinguish between these two subtypes of rhabdomyosarcoma(RMS). In the present study, we applied fluorescent in situ hybridization(FISH) and found that the Forkhead box O1(FOXO1) gene broke apart, amplified, and displayed an aneuploid signal that was related to the RMS pathological subtype.Aside from the fact that FOXO1 break-apart and its amplification were correlated with atypical ARMS, aneuploidies were usually found in atypical ERMS. In conclusion, our results detail a potential biomarker to improve the accuracy of pathological diagnosis by discriminating between atypical ARMS and atypical ERMS. | Libing Fu Yaqiong Jin Chao Jia Jie Zhang Jun Tai Hongbin Li Feng Chen Jin Shi Yongli Guo Xin Ni Lejian He | 2017 | Science China(Life Sciences)2017，60，7： | 0 |
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