Therapeutic implications of improved molecular diagnostics for rare CNS embryonal tumor entities: results of an international, retrospective study.
Katja von HoffChristine HaberlerFelix Schmitt-HoffnerElizabeth SchepkeTeresa de RojasSandra JacobsMichal ZapotockyDavid SumerauerMarta Perek-PolnikChristelle DufourDannis van VuurdenIrene SlavcJohannes GojoJessica C PicklesNicolas U GerberMaura MassiminoMaria Joao Gil-da-CostaMiklos GaramiElla KumirovaAstrid SehestedDavid ScheieOfelia CruzLucas MorenoJaeho ChoBernward ZellerNiels BovenschenMichael GrotzerDaniel AldereteMatija SnuderlOlga ZheludkovaAndrey GolanovKonstantin OkonechnikovMartin MynarekBjörn Ole JuhnkeStefan RutkowskiUlrich SchüllerBarry PizerBarbara von ZezschwitzRobert KwiecienMaximilian WechsungFrank KonietschkeEugene I HwangDominik SturmStefan M PfisterAndreas von DeimlingElisabeth J RushingMarina RyzhovaPeter HauserMaria ŁastowskaPieter WesselingFelice GiangasperoCynthia HawkinsDominique Figarella-BrangerCharles EberhartPeter BurgerMarco GessiAndrey KorshunovTom S JacquesDavid CapperTorsten PietschMarcel KoolPublished in: Neuro-oncology (2021)
The historically reported poor outcome of CNS-PNET patients becomes highly variable when tumors are molecularly classified based on DNA methylation profiling. Patients with CNS NB-FOXR2 responded well to current treatments and a standard-risk CSI-based regimen may be prospectively evaluated. The poor outcome of ETMR across applied treatment strategies substantiates the necessity for evaluation of novel treatments.