Cerebellar mutism syndrome in children with brain tumours of the posterior fossa

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Cerebellar mutism syndrome in children with brain tumours of the posterior fossa

Wibroe, Morten (författare): Rigshosp, Dept Neurosurg, Copenhagen, Denmark.;Rigshosp, Dept Pediat & Adolescent Med, Copenhagen, Denmark.,Copenhagen University Hospital

Cappelen, Johan (författare): St Olavs Hosp, Dept Neurosurg, Trondheim, Norway.,St. Olav’s University Hospital

Castor, Charlotte (författare): Lund University,Lunds universitet,Barns och familjers hälsa,Forskargrupper vid Lunds universitet,Child and Family Health,Lund University Research Groups,Skåne University Hospital

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Clausen, Niels (författare): Aarhus Univ Hosp, Dept Pediat, Aarhus, Denmark.,Aarhus University Hospital

Grillner, Pernilla (författare): Karolinska Institutet

Gudrunardottir, Thora (författare): Posterior Fossa Soc Https Www Posteriorfossa Org, Stockholm, Sweden.;North Zealand Hosp, Dept Oncol & Palliat, Hillerod, Denmark.,Posterior Fossa Society,Nordsjællands Hospital

Gupta, Ramneek (författare): Tech Univ Denmark, Ctr Biol Sequence Anal, Lyngby, Denmark.,Technical University of Denmark

Gustavsson, Bengt (författare): Karolinska Univ Hosp, Dept Neurosurg, Stockholm, Sweden.,Karolinska University Hospital

Heyman, Mats (författare): Karolinska Institutet

Holm, Stefan (författare): Karolinska Univ Sjukhuset, Dept Womens & Childrens Hlth, Stockholm, Sweden.,Karolinska University Hospital

Karppinen, Atte (författare): Helsinki Univ Hosp, Dept Neurosurg, Helsinki, Finland.,Helsinki University Central Hospital

Klausen, Camilla (författare): Rigshosp, Univ Hosp Copenhagen, Dept Neuroradiol, Copenhagen, Denmark.,Copenhagen University Hospital

Lönnqvist, Tuula (författare): Univ Helsinki, Cent Hosp, Dept Child Neurol, Helsinki, Finland.,Helsinki University Central Hospital

Mathiasen, Rene (författare): Rigshosp, Dept Pediat & Adolescent Med, Copenhagen, Denmark.,Copenhagen University Hospital

Nilsson, Pelle (författare): Uppsala universitet,Neurokirurgi,Uppsala University Hospital

Nysom, Karsten (författare): Rigshosp, Dept Pediat & Adolescent Med, Copenhagen, Denmark.,Copenhagen University Hospital

Persson, Karin (författare): Lund University,Lunds universitet,Institutionen för hälsovetenskaper,Medicinska fakulteten,Department of Health Sciences,Faculty of Medicine

Rask, Olof (författare): Lund University,Lunds universitet,Enheten för pediatrisk hematologi,Forskargrupper vid Lunds universitet,Paediatric Hematologic Research Group,Lund University Research Groups,Skåne University Hospital

Schmiegelow, Kjeld (författare): Rigshosp, Dept Pediat & Adolescent Med, Copenhagen, Denmark.;Univ Copenhagen, Inst Clin Med, Copenhagen, Denmark.;Univ Langone, Med Ctr, Perlmutter Canc Ctr, Div Pediat Hematol Oncol, New York, NY USA.,Copenhagen University Hospital,University of Copenhagen

Sehested, Astrid (författare): Rigshosp, Dept Pediat & Adolescent Med, Copenhagen, Denmark.,Copenhagen University Hospital

Thomassen, Harald (författare): St Olavs Hosp, Dept Pediat, Trondheim, Norway.,St. Olav’s University Hospital

Tonning-Olsson, Ingrid (författare): Lund University,Lunds universitet,Pediatrik, Lund,Sektion V,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Pediatrisk onkologi,Forskargrupper vid Lunds universitet,Paediatrics (Lund),Section V,Department of Clinical Sciences, Lund,Faculty of Medicine,Pediatric Oncology,Lund University Research Groups,Skåne University Hospital

Zetterqvist, Barbara (författare): Karolinska Institute

Juhler, Marianne (författare): Rigshosp, Dept Neurosurg, Copenhagen, Denmark.;Univ Copenhagen, Inst Clin Med, Copenhagen, Denmark.,University of Copenhagen,Copenhagen University Hospital

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Rigshosp, Dept Neurosurg, Copenhagen, Denmark;Rigshosp, Dept Pediat & Adolescent Med, Copenhagen, Denmark. Copenhagen University Hospital (creator_code:org_t)

2017-06-21
2017
Engelska.
Ingår i: BMC Cancer. - : BIOMED CENTRAL LTD. - 1471-2407. ; 17

Relaterad länk:: https://doi.org/10.1...; visa fler...; https://uu.diva-port... (primary) (Raw object); https://bmccancer.bi...; http://dx.doi.org/10... (free); https://urn.kb.se/re...; https://doi.org/10.1...; https://lup.lub.lu.s...; http://kipublication...; visa färre...

Tidskriftsartikel (refereegranskat)

Abstract Ämnesord

Stäng

Background: Central nervous system tumours constitute 25% of all childhood cancers; more than half are located in the posterior fossa and surgery is usually part of therapy. One of the most disabling late effects of posterior fossa tumour surgery is the cerebellar mutism syndrome (CMS) which has been reported in up to 39% of the patients but the exact incidence is uncertain since milder cases may be unrecognized. Recovery is usually incomplete. Reported risk factors are tumour type, midline location and brainstem involvement, but the exact aetiology, surgical and other risk factors, the clinical course and strategies for prevention and treatment are yet to be determined.Methods: This observational, prospective, multicentre study will include 500 children with posterior fossa tumours. It opened late 2014 with participation from 20 Nordic and Baltic centres. From 2016, five British centres and four Dutch centres will join with a total annual accrual of 130 patients. Three other major European centres are invited to join from 2016/17. Follow-up will run for 12 months after inclusion of the last patient. All patients are treated according to local practice. Clinical data are collected through standardized online registration at pre-determined time points pre- and postoperatively. Neurological status and speech functions are examined pre- operatively and postoperatively at 1-4 weeks, 2 and 12 months. Pre- and postoperative speech samples are recorded and analysed. Imaging will be reviewed centrally. Pathology is classified according to the 2007 WHO system. Germline DNA will be collected from all patients for associations between CMS characteristics and host genome variants including pathway profiles.Discussion: Through prospective and detailed collection of information on 1) differences in incidence and clinical course of CMS for different patient and tumour characteristics, 2) standardized surgical data and their association with CMS, 3) diversities and results of other therapeutic interventions, and 4) the role of host genome variants, we aim to achieve a better understanding of risk factors for and the clinical course of CMS - with the ultimate goal of defining strategies for prevention and treatment of this severely disabling condition.

Ämnesord

MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)

Nyckelord

Cancer
Paediatric
Children
Cerebellar mutism
CMS
Brain tumour
Cerebellum
Posterior fossa syndrome
Genetics
Neurosurgery
Brain tumour
Cancer
Cerebellar mutism
Cerebellum
Children
CMS
Genetics
Neurosurgery
Paediatric
Posterior fossa syndrome

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MEDICIN OCH HÄLSOVETENSKAP: MEDICIN OCH HÄLS ...; och Klinisk medicin; och Cancer och onkol ...

Artiklar i publikationen: BMC Cancer

Av lärosätet: Uppsala universitet; Lunds universitet; Karolinska Institutet

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