• Medientyp: E-Artikel
  • Titel: ETMR-10. Retrospective molecular re-evaluation of CNS PNETs; a population-based study
  • Beteiligte: Schepke, Elizabeth; Löfgren, Maja; Pietsch, Torsten; Kling, Teresia; Nordborg, Claes; Bontell, Thomas Olsson; Holm, Stefan; Öberg, Anders; Nyman, Per; Eliasson-Hofvander, Marie; Sabel, Magnus; Lannering, Birgitta; Carén, Helena
  • Erschienen: Oxford University Press (OUP), 2022
  • Erschienen in: Neuro-Oncology
  • Sprache: Englisch
  • DOI: 10.1093/neuonc/noac079.188
  • ISSN: 1522-8517; 1523-5866
  • Schlagwörter: Cancer Research ; Neurology (clinical) ; Oncology
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  • Beschreibung: <jats:title>Abstract</jats:title> <jats:p>BACKGROUND: The heterogeneous group of tumors, primitive neuro-ectodermal tumors of the central nervous system (CNS-PNETs), is a group of rare childhood embryonal tumors associated with a poor prognosis. In recent years, molecular analyzes have shown that CNS-PNETs consist of high-grade gliomas (HGG), ependymomas, different embryonal entities like atypical teratoid /rhabdoid tumors (AT/RT), CNS neuroblastoma FOXR2 and embryonal tumor with multi-layered rosettes (ETMR). Each of these tumor types is unusual and long-term clinical follow-up data are sparse. METHODS: We retrospectively re-evaluated all children (0-18 years old) diagnosed with a CNS-PNET in Sweden during 1984-2015. In total, 88 supratentorial CNS-PNETs were identified in the Swedish Childhood Cancer Registry and from these formalin-fixed paraffin-embedded tumor material was available for 69 patients. All tumors were reviewed histopathologically by an experienced neuropathologist and were analyzed using genome-wide DNA methylation profiling and classified by the MNP brain tumor classifier. RESULTS: The largest entities, after re-evaluation, were HGG (30%), CNS NB-FOXR2 (12%), AT/RT (10%) and ETMR (8%). Some tumors were difficult to classify and will be further evaluated molecularly. Some examples: Best treatment results were seen for patients with CNS-NB FOXR2 (5-year PFS: 100%) where all patients had received craniospinal radiotherapy (CSI). Patients with ETMR were all very young and survival data show early progression and poor survival (5-year OS 34%). CONCLUSIONS: Although the patient material is relatively small, it is population-based with long follow-up times. Our findings are in line with other studies and shows that CSI is important for cure for CNS-NB FOXR2 and that intensive multi-modal therapies needs to be evaluated in up-front studies for these rare embryonal tumors.</jats:p>
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