scholarly journals Treatment barriers and clinical outcome of children with medulloblastoma in China: a report from the Chinese Children’s Cancer Group (CCCG)

2021 ◽  
Author(s):  
Anthony Pak-Yin Liu ◽  
Zijun Zhen ◽  
Qunying Yang ◽  
Xiaojun Yuan ◽  
Xiaoli Ma ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Chinese Children ◽  
Treatment Barriers ◽  
Central Nervous System Tumor ◽  
Developing Regions ◽  
Cancer Group ◽  
Group 3 ◽  
System Tumor ◽  
Molecular Grouping ◽  
Risk Grouping

Abstract Background Medulloblastoma (MB) is the most common malignant central nervous system tumor of childhood. Management requires interdisciplinary care and is associated with unique challenges in developing regions. Here, we report the characteristics, clinical outcome and treatment barriers for Chinese children with MB based on a multi-institutional cohort from the Chinese Children’s Cancer Group (CCCG). Methods Retrospective cohort study among 12 Chinese pediatric oncology units from the CCCG Brain Tumor Workgroup on patients aged < 18 years diagnosed with MB from 2016-2019. Results 221 patients (male:female = 138:83) were included, 175 (79%) were ≥ 3 years of age, and 46 (21%) < 3 years. 177 patients (80%) were completely staged, among which 50 (28%) had metastasis and 70 (40%) were considered to have high-risk (HR) disease. Gross/near-total resection was achieved in 203 patients (92%). In patients where molecular grouping could be assigned, 19 (16%), 35 (29%), and 65 (54%) respectively had WNT-activated, SHH-activated, and Group 3/4 MB. The median duration between resection and initiation of adjuvant therapy was 36 days. Respective 2-year PFS and OS rates were 76.0%±3.0% and 88.0%±2.3%. PFS was significantly associated with age, metastatic status and clinical risk grouping. Chemotherapy use during CSI or alkylator choice were not significant predictors for patient outcome. Conclusions We reported the clinical profiles and outcome from the largest cohort of Chinese children with MB after multi-modal therapy. Strengths and limitations on the local provision of neuro-oncology service are identified.

scholarly journals MBRS-16. MYC REGULATED LONG NONCODING RNA LNC-HLX-2–7 IS A PUTATIVE MOLECULAR MARKER AND A THERAPEUTIC TARGET FOR GROUP 3 MEDULLOBLASTOMAS IN CHILDREN

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii401-iii401
Author(s):  
Keisuke Katsushima ◽  
Piyush Joshi ◽  
Stacie Stapleton ◽  
Alexandra Garancher ◽  
Rajeev Vibhakar ◽  
...  
Keyword(s):  
Molecular Marker ◽  
Therapeutic Target ◽  
Noncoding Rna ◽  
Tissue Microarrays ◽  
Fish Analysis ◽  
Supporting Evidence ◽  
Central Nervous System Tumor ◽  
Ffpe Samples ◽  
Group 3 ◽  
System Tumor

Abstract Medulloblastoma (MB), a central nervous system tumor that predominantly affects children, requires aggressive therapy. Recent advances in the noncoding RNA genome could contribute to the sub-classification of medulloblastoma. The focus of this study is to identify novel long noncoding RNAs (lncRNAs) as molecular markers and potential therapeutic targets within each subgroup of MBs, in particular within Group 3. We analyzed publicly available 175 RNA-seq datasets to identify a group of putative lncRNA signatures that may be able to differentiate medulloblastoma subgroups accurately. Among those, lncRNA lnc-HLX-2–7 was highly upregulated in Group 3 MB cell lines, patient-derived xenografts, FFPE samples compared to other groups. CRISPR/Cas9 deletion of the lnc-HLX-2–7 followed by the fluorescence-activated sorting and generating monoclonal Group 3 MB cells significantly reduced the cell growth and 3-D colony formation together with the induction of apoptosis. Intracranial injection to mouse cerebellum using lnc-HLX-2–7 deleted cells resulted in reduced tumor growth compared to parental cells, and tumors were further characterized by single-cell sequencing. We identified that oncogene MYC regulates lnc-HLX-2–7 and its expression can be controlled by the small molecule JQ1, a BET-bromodomain (BRD4) inhibitor that disrupts interactions with MYC. RNA-FISH analysis using FFPE, PDX, and tissue microarrays revealed that lnc-HLX-2–7 expression is specific to Group 3 MB compared to other groups. We present supporting evidence that lnc-HLX-2–7 is a novel molecular marker and a potential therapeutic target for Group 3 MBs in children.

scholarly journals Association of ST6GAL1 and CYP19A1 polymorphisms in the 3′-UTR with astrocytoma risk and prognosis in a Chinese Han population

BMC Cancer ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Tuo Wang ◽  
Yao Sun ◽  
Zichao Xiong ◽  
Jiamin Wu ◽  
Xiaoying Ding ◽  
...  
Keyword(s):  
Cox Regression ◽  
Chinese Han Population ◽  
Low Grade ◽  
Central Nervous System Tumor ◽  
Chinese Han ◽  
Cox Regression Analysis ◽  
Han Population ◽  
Potential Biomarker ◽  
Codominant Model ◽  
System Tumor

Abstract Background Astrocytoma is a common type of central nervous system tumor. In this study, we investigated the correlation between ST6GAL1 and CYP19A1 polymorphisms and the risk and prognosis of astrocytoma. Methods A total of 365 astrocytoma patients and 379 healthy controls were genotyped using the Agena MassARRAY system. The correlation between ST6GAL1 and CYP19A1 variants and astrocytoma risk was calculated using logistic regression. The survival rate of patients with astrocytoma was analyzed to evaluate prognosis. Results We found that the ST6GAL1-rs2239611 significantly decreased the risk of astrocytoma in the codominant model (p = 0.044) and dominant model (p = 0.049). In stratified analyses, CYP19A1-rs2255192 might be associated with a higher risk of astrocytoma among the low-grade subgroup under recessive (p = 0.034) and additive (p = 0.030) models. However, CYP19A1-rs4646 had a risk-decreasing effect on the high-grade subgroup in the codominant model (p = 0.044). The results of Cox regression analysis showed that the CYP19A1-rs2239611 and -rs1042757 polymorphisms were significantly correlated with the prognosis of astrocytoma. Conclusion Our results suggest that ST6GAL1 and CYP19A1 genes may be a potential biomarker of genetic susceptibility and prognosis to astrocytoma in the Chinese Han population.

scholarly journals Central liponeurocytoma: Case report and review of literature

2012 ◽  
Vol 01 (01) ◽  
pp. 083-085 ◽  
Author(s):  
Pankaj Ailawadhi ◽  
M.C. Sharma ◽  
A.K. Mahapatra ◽  
P. Sarat Chandra
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Tumor Classification ◽  
Central Nervous System Tumor ◽  
The Central Nervous System ◽  
Cerebellar Liponeurocytoma ◽  
Classification Of Tumors ◽  
System Tumor ◽  
Well Differentiated ◽  
Glioneuronal Tumors

Abstract Cerebellar liponeurocytoma consists of well-differentiated neurons with the cytology of neurocytes in addition to a population of lipidized cells. Hence it is biphasic in appearance and has been included in the category of glioneuronal tumors of the central nervous system by the WHO working group on the Classification of Tumors of the Nervous System. However, liponeurocytoma is not exclusive to the cerebellar or fourth ventricular location. Since its inclusion in the central nervous system tumor classification, nine cases with similar histological and immunohistochemical features have also been described in the lateral ventricles. We describe here such a lateral ventricular tumour in a 30-year-old woman, characteristically showing divergent glio-neuronal differentiation and lipidized neoplastic cells. Therefore, we suggest that future WHO tumor classification should consider that liponeurocytomas are not entirely restricted to the cerebellum and henceforth change of nomenclature might be considered, as also pointed out by other authors.

Hypercoaulability state in central nervous system tumor patients

1997 ◽  
Vol 99 ◽  
pp. S251
Author(s):  
M.E. Kusak ◽  
J.M. Alonso ◽  
D. Santamarta ◽  
I. Recio ◽  
J.M. Borrás ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Tumor ◽  
Tumor Patients ◽  
System Tumor

Expression of the RAG-2 gene in murine central nervous system tumor cell lines

1991 ◽  
Vol 181 (1) ◽  
pp. 151-158 ◽  
Author(s):  
Kei Tashiro ◽  
Toru Nakano ◽  
Tasuku Honjo ◽  
Tomokazu Aoki ◽  
Shin-ichi Miyatake ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Tumor Cell ◽  
Cell Lines ◽  
Central Nervous System Tumor ◽  
Tumor Cell Lines ◽  
System Tumor

scholarly journals Diagnostic accuracy of squash cytology in central nervous system tumors

2017 ◽  
Vol 20 (1) ◽  
pp. 3-9
Author(s):  
Ram Kumar Shrestha ◽  
Bibek KC ◽  
Gopal Sedain ◽  
Gita Sayami ◽  
Sushil Shilpakar ◽  
...  
Keyword(s):  
Decision Making ◽  
Central Nervous System ◽  
Nervous System ◽  
Diagnostic Accuracy ◽  
Histopathological Examination ◽  
Biological Behavior ◽  
Central Nervous System Tumor ◽  
Quick Method ◽  
System Tumor ◽  
Method Of Evaluation

Introduction: CNS tumor requires intraoperative decision making regarding the extent of tumor removal. Clinical examination and imaging studies are not sufficient enough to predict the biological behavior of the tumors. Squash cytology is a quick method of evaluation of cytomorphologic features prepared from smear technique and provide the preliminary diagnosis and aid in intraoperative decision making by differentiating neoplastic from non neoplastic and benign from malignant lesions. The aim of this study is compare the diagnostic accuracy of squash cytology to that of histopathological examination. Methods: This study consists of 36 specimens from both brain and spine subjected to both squash cytology and histopathological evaluation. The squash preparation and histopathological finding were later compared and diagnostic accuracy calculated. Results: Gliomas are the most common tumor encountered and the accuracy of Squash cytology obtained was 71%. In meningioma, 100% diagnostic accuracy was obtained however, there was limitation in accurately predict the subgroup of tumor by squash cytology alone. Other neoplastic lesions included in this study were Schwannoma, Oligodendroglioma, Ependymoma, mixed tumors and others. Overall, the accuracy predicted by squash cytology is found to be 77.8 % in this study. Conclusion: Squash cytology is rapid and reliable method of tissue diagnosis that aid in intraoperative decision making regarding the extent of Central Nervous System tumor excision

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