{"title":"儿童髓母细胞瘤的分子遗传学:诊断、治疗和预后意义","authors":"M. Doussouki, A. Gajjar, Omar Chamdine","doi":"10.2217/FNL-2018-0030","DOIUrl":null,"url":null,"abstract":"Medulloblastoma is the most common embryonal tumor in children. The current standard of care comprises surgical resection, radiation and chemotherapy. Patients are stratified into standard and high risk based on the degree of resection, presence of metastatic disease and histopathology. Cure rates dramatically improved during the past decades reaching 70–80% (high and average risk, respectively). Infant medulloblastoma has a worse outcome as the use of radiation therapy is very limited, a group of patients still has dismal outcome despite appropriate therapy, and the unacceptable long-term therapy side effects in survivors. Advanced molecular techniques have allowed scientists to discover four distinct molecular subgroups and correlate them with multiple factors such as histopathology, clinical behavior and possible therapeutic targets.","PeriodicalId":12606,"journal":{"name":"Future Neurology","volume":" ","pages":""},"PeriodicalIF":0.6000,"publicationDate":"2019-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/FNL-2018-0030","citationCount":"16","resultStr":"{\"title\":\"Molecular genetics of medulloblastoma in children: diagnostic, therapeutic and prognostic implications\",\"authors\":\"M. Doussouki, A. Gajjar, Omar Chamdine\",\"doi\":\"10.2217/FNL-2018-0030\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Medulloblastoma is the most common embryonal tumor in children. The current standard of care comprises surgical resection, radiation and chemotherapy. Patients are stratified into standard and high risk based on the degree of resection, presence of metastatic disease and histopathology. Cure rates dramatically improved during the past decades reaching 70–80% (high and average risk, respectively). Infant medulloblastoma has a worse outcome as the use of radiation therapy is very limited, a group of patients still has dismal outcome despite appropriate therapy, and the unacceptable long-term therapy side effects in survivors. Advanced molecular techniques have allowed scientists to discover four distinct molecular subgroups and correlate them with multiple factors such as histopathology, clinical behavior and possible therapeutic targets.\",\"PeriodicalId\":12606,\"journal\":{\"name\":\"Future Neurology\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":0.6000,\"publicationDate\":\"2019-02-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://sci-hub-pdf.com/10.2217/FNL-2018-0030\",\"citationCount\":\"16\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Future Neurology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.2217/FNL-2018-0030\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Future Neurology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.2217/FNL-2018-0030","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Molecular genetics of medulloblastoma in children: diagnostic, therapeutic and prognostic implications
Medulloblastoma is the most common embryonal tumor in children. The current standard of care comprises surgical resection, radiation and chemotherapy. Patients are stratified into standard and high risk based on the degree of resection, presence of metastatic disease and histopathology. Cure rates dramatically improved during the past decades reaching 70–80% (high and average risk, respectively). Infant medulloblastoma has a worse outcome as the use of radiation therapy is very limited, a group of patients still has dismal outcome despite appropriate therapy, and the unacceptable long-term therapy side effects in survivors. Advanced molecular techniques have allowed scientists to discover four distinct molecular subgroups and correlate them with multiple factors such as histopathology, clinical behavior and possible therapeutic targets.
期刊介绍:
The neurological landscape is changing rapidly. From the technological perspective, advanced molecular approaches and imaging modalities have greatly increased our understanding of neurological disease, with enhanced prospects for effective treatments in common but very serious disorders such as stroke, epilepsy, multiple sclerosis and Parkinson’s disease. Nevertheless, at the same time, the healthcare community is increasingly challenged by the rise in neurodegenerative diseases consequent upon demographic changes in developed countries.