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000131487 1001_ $$aAndreiuolo, Felipe$$b0
000131487 245__ $$aIntegrating Tenascin-C protein expression and 1q25 copy number status in pediatric intracranial ependymoma prognostication: A new model for risk stratification.
000131487 260__ $$aLawrence, Kan.$$bPLoS$$c2017
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000131487 520__ $$aDespite multimodal therapy, prognosis of pediatric intracranial ependymomas remains poor with a 5-year survival rate below 70% and frequent late deaths.This multicentric European study evaluated putative prognostic biomarkers. Tenascin-C (TNC) immunohistochemical expression and copy number status of 1q25 were retained for a pooled analysis of 5 independent cohorts. The prognostic value of TNC and 1q25 on the overall survival (OS) was assessed using a Cox model adjusted to age at diagnosis, tumor location, WHO grade, extent of resection, radiotherapy and stratified by cohort. Stratification on a predictor that did not satisfy the proportional hazards assumption was considered. Model performance was evaluated and an internal-external cross validation was performed.Among complete cases with 5-year median follow-up (n = 470; 131 deaths), TNC and 1q25 gain were significantly associated with age at diagnosis and posterior fossa tumor location. 1q25 status added independent prognostic value for death beyond the classical variables with a hazard ratio (HR) = 2.19 95%CI = [1.29; 3.76] (p = 0.004), while TNC prognostic relation was tumor location-dependent with HR = 2.19 95%CI = [1.29; 3.76] (p = 0.004) in posterior fossa and HR = 0.64 [0.28; 1.48] (p = 0.295) in supratentorial (interaction p value = 0.015). The derived prognostic score identified 3 different robust risk groups. The omission of upfront RT was not associated with OS for good and intermediate prognostic groups while the absence of upfront RT was negatively associated with OS in the poor risk group.Integrated TNC expression and 1q25 status are useful to better stratify patients and to eventually adapt treatment regimens in pediatric intracranial ependymoma.
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000131487 650_7 $$2NLM Chemicals$$aTenascin
000131487 7001_ $$aLe Teuff, Gwénaël$$b1
000131487 7001_ $$aBayar, Mohamed Amine$$b2
000131487 7001_ $$aKilday, John-Paul$$b3
000131487 7001_ $$aPietsch, Torsten$$b4
000131487 7001_ $$avon Bueren, André O$$b5
000131487 7001_ $$0P:(DE-He78)046fd145f1008f83f6236580727bbc0f$$aWitt, Hendrik$$b6$$udkfz
000131487 7001_ $$0P:(DE-He78)8d9c904a6cea14d4c99c78ba46e41f93$$aKorshunov, Andrey$$b7$$udkfz
000131487 7001_ $$aModena, Piergiorgio$$b8
000131487 7001_ $$0P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aPfister, Stefan$$b9$$udkfz
000131487 7001_ $$aPagès, Mélanie$$b10
000131487 7001_ $$aCastel, David$$b11
000131487 7001_ $$aGiangaspero, Felice$$b12
000131487 7001_ $$aChimelli, Leila$$b13
000131487 7001_ $$aVarlet, Pascale$$b14
000131487 7001_ $$aRutkowski, Stefan$$b15
000131487 7001_ $$aFrappaz, Didier$$b16
000131487 7001_ $$aMassimino, Maura$$b17
000131487 7001_ $$aGrundy, Richard$$b18
000131487 7001_ $$aGrill, Jacques$$b19
000131487 7001_ $$aBIOMECA, SIOP Ependymoma Biology Working Group$$b20$$eCollaboration Author
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