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Supratentorial CNS-PNETs in children; a Swedish population-based study with molecular re-evaluation and long-term follow-up
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- Schepke, Elizabeth (författare)
- Gothenburg University,Göteborgs universitet,Institutionen för biomedicin, avdelningen för medicinsk kemi och cellbiologi,Sahlgrenska Centrum för Cancerforskning (SCCR),Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology,Sahlgrenska Center for Cancer Research (SCCR),Sahlgrens Univ Hosp, Queen Silv Childrens Hosp, Childhood Canc Ctr, Gothenburg, Sweden.;Univ Gothenburg, Sahlgrenska Acad, Sahlgrenska Ctr Canc Res, Inst Biomed,Dept Med Biochem & Cell Biol, Medicinaregatan 1F, S-40530 Gothenburg, Sweden.
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- Löfgren, Maja (författare)
- Gothenburg University,Göteborgs universitet,Sahlgrenska Centrum för Cancerforskning (SCCR),Institutionen för biomedicin, avdelningen för medicinsk kemi och cellbiologi,Sahlgrenska Center for Cancer Research (SCCR),Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology,Univ Gothenburg, Sahlgrenska Acad, Sahlgrenska Ctr Canc Res, Inst Biomed,Dept Med Biochem & Cell Biol, Medicinaregatan 1F, S-40530 Gothenburg, Sweden.
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- Pietsch, T. (författare)
- Univ Bonn Med Ctr, DGNN Brain Tumour Reference Ctr, Dept Neuropathol, Bonn, Germany.
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- 2023-03-09
- 2023
- Engelska.
- Ingår i: Clinical Epigenetics. - : Springer Science and Business Media LLC. - 1868-7075 .- 1868-7083. ; 15:1
- Tidskriftsartikel (refereegranskat)
Abstract
Ämnesord
Stäng
- BackgroundMolecular analyses have shown that tumours diagnosed as supratentorial primitive neuro-ectodermal tumours of the central nervous system (CNS-PNETs) in the past represent a heterogenous group of rare childhood tumours including high-grade gliomas (HGG), ependymomas, atypical teratoid/rhabdoid tumours (AT/RT), CNS neuroblastoma with forkhead box R2 (FOXR2) activation and embryonal tumour with multi-layered rosettes (ETMR). All these tumour types are rare and long-term clinical follow-up data are sparse. We retrospectively re-evaluated all children (0-18 years old) diagnosed with a CNS-PNET in Sweden during 1984-2015 and collected clinical data.MethodsIn total, 88 supratentorial CNS-PNETs were identified in the Swedish Childhood Cancer Registry and from these formalin-fixed paraffin-embedded tumour material was available for 71 patients. These tumours were histopathologically re-evaluated and, in addition, analysed using genome-wide DNA methylation profiling and classified by the MNP brain tumour classifier.ResultsThe most frequent tumour types, after histopathological re-evaluation, were HGG (35%) followed by AT/RT (11%), CNS NB-FOXR2 (10%) and ETMR (8%). DNA methylation profiling could further divide the tumours into specific subtypes and with a high accuracy classify these rare embryonal tumours. The 5 and 10-year overall survival (OS) for the whole CNS-PNET cohort was 45% +/- 12% and 42% +/- 12%, respectively. However, the different groups of tumour types identified after re-evaluation displayed very variable survival patterns, with a poor outcome for HGG and ETMR patients with 5-year OS 20% +/- 16% and 33% +/- 35%, respectively. On the contrary, high PFS and OS was observed for patients with CNS NB-FOXR2 (5-year 100% for both). Survival rates remained stable even after 15-years of follow-up.ConclusionsOur findings demonstrate, in a national based setting, the molecular heterogeneity of these tumours and show that DNA methylation profiling of these tumours provides an indispensable tool in distinguishing these rare tumours. Long-term follow-up data confirms previous findings with a favourable outcome for CNS NB-FOXR2 tumours and poor chances of survival for ETMR and HGG.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Kirurgi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Surgery (hsv//eng)
Nyckelord
- CNS-PNET
- Epigenetics
- DNA methylation profiling
- CNS NB-FOXR2
- ETMR
- Long term survival
- CNS-PNET
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