FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3111637277> ?p ?o ?g. }

• W3111637277 endingPage "iii446" @default.
• W3111637277 startingPage "iii445" @default.
• W3111637277 abstract "Abstract BACKGROUND Constitutional mismatch repair deficiency syndrome (CMMRD) is a severe cancer predisposition syndrome resulting in early onset central nervous system (CNS) and other cancers. International guidelines for surveillance exist but no study has systematically evaluated the efficacy of this protocol. METHODS We surveyed all confirmed CMMRD patients in the International Replication Repair Deficiency Consortium. A surveillance protocol consisting of frequent biochemical, endoscopic and imaging (CNS and total body MRI) studies were employed. Survival analyses and efficacy of each method were assessed. RESULTS Surveillance data were collected from 105 CMMRD individuals from 41 countries. Of the 193 malignant tumors, CNS malignancies were the most common (44%). The surveillance protocol uncovered 49 asymptomatic tumors including 16 glioblastomas and medulloblastomas. Five-year overall survival was 89% for tumors discovered by surveillance, and 61% for symptomatic tumors (p&lt;0.004). Similarly, 5-year survival was 82+/-11% and 24+/-6% for surveillance and non-surveillance of brain tumors (p=0.005). Yearly total body and q6 month brain MRI detected asymptomatic cancers in all but 3 symptomatic CNS gliomas. These were tumors uncovered when time between scans was &gt;6 months as per protocol. Finally, of the low grade tumors identified asymptomatically, 5 were low grade gliomas. All of the low grade gliomas, which were not resected transformed to high grade tumors at a median of 1.6 ± 0.9 years. CONCLUSION These data support a survival benefit in CMMRD patients undergoing a surveillance protocol. Adherence to protocol and resection of lower grade lesions may improve survival for patients with CNS tumors." @default.
• W3111637277 created "2020-12-21" @default.
• W3111637277 creator A5000685371 @default.
• W3111637277 creator A5000990352 @default.
• W3111637277 creator A5001293585 @default.
• W3111637277 creator A5001294228 @default.
• W3111637277 creator A5001500764 @default.
• W3111637277 creator A5001673025 @default.
• W3111637277 creator A5001680618 @default.
• W3111637277 creator A5002444356 @default.
• W3111637277 creator A5002485541 @default.
• W3111637277 creator A5004091287 @default.
• W3111637277 creator A5008397335 @default.
• W3111637277 creator A5008697710 @default.
• W3111637277 creator A5010541899 @default.
• W3111637277 creator A5010939758 @default.
• W3111637277 creator A5011610183 @default.
• W3111637277 creator A5012665304 @default.
• W3111637277 creator A5012891627 @default.
• W3111637277 creator A5012955659 @default.
• W3111637277 creator A5013343796 @default.
• W3111637277 creator A5013395863 @default.
• W3111637277 creator A5013778862 @default.
• W3111637277 creator A5016712043 @default.
• W3111637277 creator A5018418303 @default.
• W3111637277 creator A5018466923 @default.
• W3111637277 creator A5019318531 @default.
• W3111637277 creator A5019468881 @default.
• W3111637277 creator A5022310695 @default.
• W3111637277 creator A5022912254 @default.
• W3111637277 creator A5025442855 @default.
• W3111637277 creator A5027426012 @default.
• W3111637277 creator A5029328183 @default.
• W3111637277 creator A5032789191 @default.
• W3111637277 creator A5033045382 @default.
• W3111637277 creator A5033190757 @default.
• W3111637277 creator A5033692158 @default.
• W3111637277 creator A5036323261 @default.
• W3111637277 creator A5037614274 @default.
• W3111637277 creator A5039736511 @default.
• W3111637277 creator A5040944524 @default.
• W3111637277 creator A5041074495 @default.
• W3111637277 creator A5042286173 @default.
• W3111637277 creator A5042455579 @default.
• W3111637277 creator A5043108586 @default.
• W3111637277 creator A5043812430 @default.
• W3111637277 creator A5043859307 @default.
• W3111637277 creator A5044218928 @default.
• W3111637277 creator A5046262164 @default.
• W3111637277 creator A5046321083 @default.
• W3111637277 creator A5046351275 @default.
• W3111637277 creator A5046496883 @default.
• W3111637277 creator A5046879142 @default.
• W3111637277 creator A5047012744 @default.
• W3111637277 creator A5048228345 @default.
• W3111637277 creator A5051455843 @default.
• W3111637277 creator A5052730233 @default.
• W3111637277 creator A5054237580 @default.
• W3111637277 creator A5054848470 @default.
• W3111637277 creator A5061254573 @default.
• W3111637277 creator A5062106831 @default.
• W3111637277 creator A5062198630 @default.
• W3111637277 creator A5063922230 @default.
• W3111637277 creator A5067440904 @default.
• W3111637277 creator A5067473120 @default.
• W3111637277 creator A5067713698 @default.
• W3111637277 creator A5068257880 @default.
• W3111637277 creator A5068784960 @default.
• W3111637277 creator A5071421450 @default.
• W3111637277 creator A5072241415 @default.
• W3111637277 creator A5073229796 @default.
• W3111637277 creator A5075183737 @default.
• W3111637277 creator A5075401861 @default.
• W3111637277 creator A5076649152 @default.
• W3111637277 creator A5078418332 @default.
• W3111637277 creator A5079656701 @default.
• W3111637277 creator A5082885008 @default.
• W3111637277 creator A5083628894 @default.
• W3111637277 creator A5084123340 @default.
• W3111637277 creator A5084930644 @default.
• W3111637277 creator A5084984653 @default.
• W3111637277 creator A5086737230 @default.
• W3111637277 creator A5086914791 @default.
• W3111637277 creator A5090555697 @default.
• W3111637277 creator A5091712536 @default.
• W3111637277 creator A5091756510 @default.
• W3111637277 date "2020-12-01" @default.
• W3111637277 modified "2023-10-13" @default.
• W3111637277 title "RARE-17. SURVIVAL BENEFIT FOR INDIVIDUALS WITH CONSTITUTIONAL MISMATCH REPAIR DEFICIENCY SYNDROME AND BRAIN TUMORS WHO UNDERGO SURVEILLANCE PROTOCOL. A REPORT FROM THE INTERNATIONAL REPLICATION REPAIR CONSORTIUM" @default.
• W3111637277 doi "https://doi.org/10.1093/neuonc/noaa222.728" @default.
• W3111637277 hasPublicationYear "2020" @default.
• W3111637277 type Work @default.
• W3111637277 sameAs 3111637277 @default.
• W3111637277 citedByCount "0" @default.
• W3111637277 crossrefType "journal-article" @default.
• W3111637277 hasAuthorship W3111637277A5000685371 @default.
• W3111637277 hasAuthorship W3111637277A5000990352 @default.
• W3111637277 hasAuthorship W3111637277A5001293585 @default.

• next