Glioma Pub Date : 2020-07-01 DOI: 10.4103/glioma.glioma_24_20

G. Wong, K. Li, M. Poon, H. Ng

{"title":"Is adult medulloblastoma merely the counterpart of pediatric medulloblastoma?","authors":"G. Wong, K. Li, M. Poon, H. Ng","doi":"10.4103/glioma.glioma_24_20","DOIUrl":"https://doi.org/10.4103/glioma.glioma_24_20","url":null,"abstract":"Medulloblastoma is one of the most common pediatric malignant brain tumors. Its understanding and treatment have advanced rapidly over the decade, with the identification of four distinct molecular groups. In contrast, adult medulloblastoma is a rare entity that accounts for <1% of adult central nervous system tumors, and is understudied in both its genetic landscape and clinical management. Adult medulloblastomas demonstrate many differences from pediatric medulloblastomas that are relevant to clinicians and biologists. Unlike its pediatric counterpart, adult medulloblastomas are typically located laterally in the cerebellum, are seldom metastatic, and commonly relapse beyond 5 years. The distribution and survival outcomes of molecular groups in adult medulloblastoma differ from those in pediatric medulloblastoma, with the sonic hedgehog-activated group being the predominant and most well-studied group in adults. Adult medulloblastomas also exhibit cytogenetic and mutational characteristics unique to this age group, such as the high frequency of telomerase reverse transcriptase promoter mutations and the paucity of MYC and MYCN amplifications. Clinical trials for adult medulloblastoma need to take into account the clinical and biological differences between adult and pediatric medulloblastomas, for example through the use of smoothened inhibitors in adult SHH medulloblastomas to lower the toxicities resulting from direct adoption of pediatric chemotherapeutic regimens. This review summarizes the clinical characteristics, molecular groups, genetic features, and treatment of adult medulloblastoma, with a focus on its differences from pediatric medulloblastoma.","PeriodicalId":12731,"journal":{"name":"Glioma","volume":"3 1","pages":"90 - 96"},"PeriodicalIF":0.0,"publicationDate":"2020-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44706466","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Comprehensive RNAseq analysis reveals PIK3CD promotes glioblastoma tumorigenesis by mediating PI3K-Akt signaling pathway 综合RNAseq分析发现，PIK3CD通过介导PI3K-Akt信号通路促进胶质母细胞瘤的发生

Glioma Pub Date : 2020-07-01 DOI: 10.4103/glioma.glioma_23_20

Zulfikar Azam, W. Shao, H. Ng, Jing Wang, Zhongping Chen, S. To

{"title":"Comprehensive RNAseq analysis reveals PIK3CD promotes glioblastoma tumorigenesis by mediating PI3K-Akt signaling pathway","authors":"Zulfikar Azam, W. Shao, H. Ng, Jing Wang, Zhongping Chen, S. To","doi":"10.4103/glioma.glioma_23_20","DOIUrl":"https://doi.org/10.4103/glioma.glioma_23_20","url":null,"abstract":"Background and Aim: Glioblastoma (GBM) is the most common and aggressive form of primary malignant brain tumors. Phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit delta (PIK3CD), which is overexpressed in GBM, is involved in GBM pathogenesis and drug resistance. However, the molecular mechanism by which PIK3CD drives its transcriptional program toward GBM favors remains elusive. Materials and Methods: Clustered Regularly Interspaced Short Palindromic Repeats (CRISPR) and CRISPR-associated 9 technology was used to knock-out (KO) PIK3CD gene, and comprehensive RNAseq analysis was performed to investigate the underlying role of PIK3CD in GBM. Results: To minimize the off-target effects, two KO cell clones were used, and our data showed that PIK3CD KO altered the expression 306 genes in both KO cell clones compared with the parent U87 cell line. Gene set enrichment analysis revealed that genes involved in epithelial-mesenchymal (MES) transition-related biological processes were highly depressed in both KO cell clones in a similar fashion, suggesting PIK3CD's involvement in MES transformation/transition in GBM. Comprehensive pathway analysis by three different platforms confirmed that PIK3CD exerted its oncogenic function in GBM through phosphatidylinositol 3-kinase (PI3K)-Akt signaling pathway. In addition, other signaling pathways (integrin, cadherin, Wnt, and inflammation mediated by chemokine and cytokine signaling pathways) were found decreased in the KO cell clones. Further, The Cancer Genomic Atlas (TCGA) analysis of our PI3K-Akt pathway-related genes showed a similar pattern of expression. Conclusion: PIK3CD is involved in GBM pathogenesis, and this action probably mediated through the PI3K-Akt signaling pathway.","PeriodicalId":12731,"journal":{"name":"Glioma","volume":"3 1","pages":"135 - 142"},"PeriodicalIF":0.0,"publicationDate":"2020-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46161086","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Strategy of awake surgical resection for glioma based on intraoperative functional mapping and monitoring: A case report 基于术中功能定位和监测的神经胶质瘤清醒手术切除策略:1例报告

Glioma Pub Date : 2020-07-01 DOI: 10.4103/glioma.glioma_15_20

Ying-wu Shi, S. Ge, P. Ji, Jing-hui Liu, Yuan Wang, Shao-chun Guo, Y. Zhai, Min Chao, G. Gao, Y. Qu, Liang Wang

引用次数: 0

Advancement of clinical therapeutic research on glioma: A narrative review 胶质瘤临床治疗研究进展综述

Glioma Pub Date : 2020-07-01 DOI: 10.4103/glioma.glioma_18_20

Fengping Li, Chao Ma, Cheng-shi Xu, Zhi-yong Pan, Zhiqiang Li

引用次数: 1

Maximal safe resection of diffuse low-grade gliomas within/near motor areas using awake craniotomy with intraoperative cortical/subcortical mapping via direct electrical stimulation: A narrative review 通过直接电刺激进行清醒开颅术术中皮质/皮质下定位，最大限度地安全切除运动区内/附近的弥漫性低级别胶质瘤:一篇叙述性综述

Glioma Pub Date : 2020-07-01 DOI: 10.4103/glioma.glioma_14_20

Ruixin Yang, Hongmin Bai

{"title":"Maximal safe resection of diffuse low-grade gliomas within/near motor areas using awake craniotomy with intraoperative cortical/subcortical mapping via direct electrical stimulation: A narrative review","authors":"Ruixin Yang, Hongmin Bai","doi":"10.4103/glioma.glioma_14_20","DOIUrl":"https://doi.org/10.4103/glioma.glioma_14_20","url":null,"abstract":"Diffuse low-grade gliomas are a diverse category of neuroepithelial neoplasms, which are characterized by a low proliferation index and an indolent course with a long-term survival in comparison with high-grade gliomas. It is convinced that maximal safe resection can significantly increase survival. Multiple intraoperative techniques help neurosurgeons to maximize the resection with a safe range; however, the tumors involving motor areas are still intractable for many surgeons who believe that these tumors are unresectable. We searched on PubMed database and summarized studies and reviews about diffuse low-grade gliomas within/near motor areas. Moreover, studies about anatomy about motor area were also reviewed. In this article, we discussed the anatomy of the central lobe and supplementary motor area, including the cortex, subcortical fibers, and relevant vessels, as well as the technical details of awake craniotomy and direct electrical stimulation. At last, combined with some cases, we try to demonstrate that tumors within/near motor areas are resectable, which may cause only mild neurological deficits using awake craniotomy with intraoperative monitoring by cortical/subcortical mapping, and, furthermore, it provides a longer and better survival for those young patients.","PeriodicalId":12731,"journal":{"name":"Glioma","volume":"3 1","pages":"126 - 134"},"PeriodicalIF":0.0,"publicationDate":"2020-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46064864","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Mechanisms of telomere maintenance in pediatric brain tumors: Promising targets for therapy – A narrative review 儿童脑肿瘤的端粒维持机制:有希望的治疗靶点

Glioma Pub Date : 2020-07-01 DOI: 10.4103/glioma.glioma_20_20

S. Minasi, F. Gianno, Hiba Alzoubi, M. Antonelli, F. Giangaspero, F. Buttarelli

{"title":"Mechanisms of telomere maintenance in pediatric brain tumors: Promising targets for therapy – A narrative review","authors":"S. Minasi, F. Gianno, Hiba Alzoubi, M. Antonelli, F. Giangaspero, F. Buttarelli","doi":"10.4103/glioma.glioma_20_20","DOIUrl":"https://doi.org/10.4103/glioma.glioma_20_20","url":null,"abstract":"Recent advances in genetic and molecular characterization of telomere maintenance mechanisms (TMMs) highlighted their strong relationship with cancer pathogenesis; neoplastic cells rely on two mechanisms to maintain telomere length and escape from replicative senescence: (a) reactivation of telomerase expression and (b) activation of alternative lengthening of telomere (ALT). Our aims are to describe the role of telomere maintenance in the context of recently published literature regarding pediatric brain cancers and to discuss the emerging therapeutic strategies to target telomerase-positive and ALT-positive tumors. In this review, we illustrate the incidence of TMM via telomerase or ALT and discuss the importance of analyzing telomere length and ALT-associated genetic alterations in certain histological/molecular subtypes of pediatric brain tumors, as potential therapeutic biomarkers. Telomerase-dependent TMM is a common mechanism in SHH-medulloblastomas and ependymomas, which could potentially benefit from antitelomerase therapies, while ALT-dependent TMM is more frequently activated in α-thalassemia/mental retardation syndrome X-linked/H3.3-mutated pediatric high-grade gliomas, metastatic medulloblastomas, and choroid plexus tumors, which could potentially be treated with ALT-targeted drugs. Conversely, pediatric low-grade gliomas lack both mechanisms of telomere maintenance, and anti-TMM therapies do not appear to be a promising strategy for these tumors.","PeriodicalId":12731,"journal":{"name":"Glioma","volume":"3 1","pages":"105 - 118"},"PeriodicalIF":0.0,"publicationDate":"2020-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"70735422","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

To do genomics or not do? This is the question 做基因组学还是不做?这就是问题所在

Glioma Pub Date : 2020-07-01 DOI: 10.4103/glioma.glioma_22_20

H. Ng, A. Chan, Nim-Chi Amanda Kan, Dennis Ku, D. Chan, K. Li

引用次数: 1

Carbonic anhydrase IX as a marker of hypoxia in gliomas: A narrative review 碳酸酐酶IX作为胶质瘤缺氧标志物的研究进展

Glioma Pub Date : 2020-07-01 DOI: 10.4103/glioma.glioma_19_20

R. McLendon

引用次数: 1

Functional remodeling of brain language networks 大脑语言网络的功能重塑

Glioma Pub Date : 2020-04-01 DOI: 10.4103/glioma.glioma_12_20

N. Hameed, Jinsong Wu

引用次数: 0

A young adult patient with Li-Fraumeni syndrome-associated glioblastoma: Case discussion and literature review 一例年轻成人李-弗劳梅尼综合征相关胶质母细胞瘤患者：病例讨论和文献复习

Glioma Pub Date : 2020-04-01 DOI: 10.4103/glioma.glioma_17_20

Xiao-Yu Wu, S. Tian, Biling Liang, Qunying Yang, H. Ng, Shao‐xiong Wu, Q. Chang, Zhongping Chen

{"title":"A young adult patient with Li-Fraumeni syndrome-associated glioblastoma: Case discussion and literature review","authors":"Xiao-Yu Wu, S. Tian, Biling Liang, Qunying Yang, H. Ng, Shao‐xiong Wu, Q. Chang, Zhongping Chen","doi":"10.4103/glioma.glioma_17_20","DOIUrl":"https://doi.org/10.4103/glioma.glioma_17_20","url":null,"abstract":"Li-Fraumeni syndrome is an autosomal dominant cancer predisposition syndrome caused by germ line alterations in the tumor suppressor gene TP53, with an incidence of 1 in 5000–1 in 20,000. Li-Fraumeni syndrome is associated with numerous malignancies, including astrocytoma. Here, we report the case of a female patient diagnosed with glioblastoma in the right temporal lobe at the age of 22 years. She was treated with surgery followed by radiation and chemotherapy and achieved a complete response. Not surprisingly, the patient relapsed 7 years later and underwent a second surgery and radiation concurrent with temozolomide followed by chemotherapy with various agents. The patient currently remains tumor-free. Genetic testing revealed that the tumor contained a germ line mutation of TP53 (p.R282W). Pertinent family history included a mother who suffered from leukemia. Therefore, given the patient's medical and family history, we consider this is a case of Li-Fraumeni syndrome associated with glioblastoma. The ethics approval is not applied since the case was in consultation with experts arranged through meeting organizer.","PeriodicalId":12731,"journal":{"name":"Glioma","volume":"3 1","pages":"71 - 75"},"PeriodicalIF":0.0,"publicationDate":"2020-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49563792","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

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