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Conference Paper: Epidemiology, management and outcome of childhood Atypical Teratoid/Rhabdoid Tumours (ATRT) of central nervous system

TitleEpidemiology, management and outcome of childhood Atypical Teratoid/Rhabdoid Tumours (ATRT) of central nervous system
Authors
Issue Date2016
PublisherOxford University Press. The Journal's web site is located at http://neuro-oncology.dukejournals.org
Citation
The 17th International Symposium on Pediatric Neuro-Oncology (ISPNO 2016), Liverpool, UK., 12-15 June 2016. In Neuro-Oncology, 2016, v. 18 suppl. 3, abstract no. AT-17 How to Cite?
AbstractBACKGROUND: Atypical Teratoid Rhabdoid Tumour (ATRT) is a rare and aggressive brain tumour (WHO Grade IV) in young children which carries poor prognosis. We reviewed our clinical experience for these patients. METHOD: Retrospective review of children diagnosed with CNS ATRT from Jan 1999 to Dec 2014 treated in five hospitals in Hong Kong covering almost all cases. RESULT: Fifteen patients (N = 15, 7 boys and 8 girls) were diagnosed with CNS ATRT within 16 years. The median age was 28 months (range 2.4-170.4) and 73% were younger than 36 months. Primary tumours were located at supratentorium (n = 7), posterior fossa (n = 7) and spine (n = 1). Two patients (13%) had metastasis. Eight specimens had confirmed loss of INI-1 expression. Germline SMARCB1 mutation was tested in 3 patients and were negative. Gross total resection was achieved in 11 patients (73%). Eleven patients received chemotherapy regimens: Carbo/VCR/Ifos/VP16 (36%), Doxo/Ifos/Carbo/VP16/VCR/CPM/Dact (18%), Doxo/Dact/CDDP/VCR/CCNU (36%), CDDP/CCNU/VCR (9%). High-dose chemotherapy was given in 2 patients (18%). Eight patients (53%) received upfront radiotherapy at median age of 36 months. The median survival was 10.8 months while the 2 years and 5 years event-free survival were 30% and 15% respectively by Kaplan-Meier analysis. We had 4 survivors who underwent gross total resection, systemic and intrathecal/intra-ventricular chemotherapy with upfront radiotherapy. They were followed up for 1.5 to 9.7 years, of which three were disease-free while 1 had residual disease. CONCLUSION: CNS ATRT carries poor prognosis despite intensive treatment in young children. Some patients survived after multidisciplinary intensive treatment.
DescriptionThis journal suppl. entitled: Abstracts from the 17th International Symposium on Pediatric Neuro-Oncology (ISPNO), June 12-15, 2016, Liverpool, United Kingdom
Persistent Identifierhttp://hdl.handle.net/10722/226505
ISSN
2015 Impact Factor: 7.371
2015 SCImago Journal Rankings: 3.196

 

DC FieldValueLanguage
dc.contributor.authorKu, DTL-
dc.contributor.authorShing, MMK-
dc.contributor.authorChang, KO-
dc.contributor.authorLiu, APY-
dc.contributor.authorLing, SK-
dc.contributor.authorTo, KF-
dc.contributor.authorChan, GCF-
dc.date.accessioned2016-06-17T07:44:35Z-
dc.date.available2016-06-17T07:44:35Z-
dc.date.issued2016-
dc.identifier.citationThe 17th International Symposium on Pediatric Neuro-Oncology (ISPNO 2016), Liverpool, UK., 12-15 June 2016. In Neuro-Oncology, 2016, v. 18 suppl. 3, abstract no. AT-17-
dc.identifier.issn1522-8517-
dc.identifier.urihttp://hdl.handle.net/10722/226505-
dc.descriptionThis journal suppl. entitled: Abstracts from the 17th International Symposium on Pediatric Neuro-Oncology (ISPNO), June 12-15, 2016, Liverpool, United Kingdom-
dc.description.abstractBACKGROUND: Atypical Teratoid Rhabdoid Tumour (ATRT) is a rare and aggressive brain tumour (WHO Grade IV) in young children which carries poor prognosis. We reviewed our clinical experience for these patients. METHOD: Retrospective review of children diagnosed with CNS ATRT from Jan 1999 to Dec 2014 treated in five hospitals in Hong Kong covering almost all cases. RESULT: Fifteen patients (N = 15, 7 boys and 8 girls) were diagnosed with CNS ATRT within 16 years. The median age was 28 months (range 2.4-170.4) and 73% were younger than 36 months. Primary tumours were located at supratentorium (n = 7), posterior fossa (n = 7) and spine (n = 1). Two patients (13%) had metastasis. Eight specimens had confirmed loss of INI-1 expression. Germline SMARCB1 mutation was tested in 3 patients and were negative. Gross total resection was achieved in 11 patients (73%). Eleven patients received chemotherapy regimens: Carbo/VCR/Ifos/VP16 (36%), Doxo/Ifos/Carbo/VP16/VCR/CPM/Dact (18%), Doxo/Dact/CDDP/VCR/CCNU (36%), CDDP/CCNU/VCR (9%). High-dose chemotherapy was given in 2 patients (18%). Eight patients (53%) received upfront radiotherapy at median age of 36 months. The median survival was 10.8 months while the 2 years and 5 years event-free survival were 30% and 15% respectively by Kaplan-Meier analysis. We had 4 survivors who underwent gross total resection, systemic and intrathecal/intra-ventricular chemotherapy with upfront radiotherapy. They were followed up for 1.5 to 9.7 years, of which three were disease-free while 1 had residual disease. CONCLUSION: CNS ATRT carries poor prognosis despite intensive treatment in young children. Some patients survived after multidisciplinary intensive treatment.-
dc.languageeng-
dc.publisherOxford University Press. The Journal's web site is located at http://neuro-oncology.dukejournals.org-
dc.relation.ispartofNeuro-Oncology-
dc.titleEpidemiology, management and outcome of childhood Atypical Teratoid/Rhabdoid Tumours (ATRT) of central nervous system-
dc.typeConference_Paper-
dc.identifier.emailLiu, APY: apyliu@hku.hk-
dc.identifier.emailChan, GCF: gcfchan@hku.hk-
dc.identifier.authorityLiu, APY=rp01357-
dc.identifier.authorityChan, GCF=rp00431-
dc.description.naturelink_to_OA_fulltext-
dc.identifier.doi10.1093/neuonc/now065.16-
dc.identifier.hkuros258399-
dc.identifier.volume18-
dc.identifier.issuesuppl. 3, abstract no. AT-17-
dc.publisher.placeUnited States-
dc.customcontrol.immutablesml 160817-

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