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000172531 0247_ $$2doi$$a10.1093/neuonc/noab230
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000172531 0247_ $$2ISSN$$a1523-5866
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000172531 037__ $$aDKFZ-2021-02079
000172531 041__ $$aEnglish
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000172531 1001_ $$aMassimino, Maura$$b0
000172531 245__ $$aTreatment and outcome of intracranial ependymoma after first relapse in AIEOP 2 nd protocol.
000172531 260__ $$aOxford$$bOxford Univ. Press$$c2022
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000172531 500__ $$a Volume 24, Issue 3, March 2022, Pages 467–479
000172531 520__ $$aMore than 40% of patients with intracranial ependymoma need a salvage treatment within 5 years after diagnosis, and no standard treatment is available as yet. We report the outcome after first relapse of 64 patients treated within the 2 nd AIEOP protocol.We considered relapse sites and treatments ,i.e. various combinations of complete/incomplete surgery, if followed by standard or hypo-fractionated radiation(RT) ± chemotherapy(CT). Molecular analyses were available for 38/64 samples obtained at first diagnosis. Of the 64 cases, 55 were suitable for subsequent analyses.The median follow-up was 147 months after diagnosis, 84 after first relapse, 5-year EFS/OS were 26.2%/30.8% (median EFS/OS 13/32 months) after relapse. For patients with a local relapse(LR), the 5-year cumulative incidence of second LRs was 51.6%, with a 5-year event-specific probability of being LR-free of 40.0%. Tumor site/grade, need for shunting, age above/below 3 years, molecular subgroup at diagnosis, had no influence on outcomes. Due to variation in the RT dose/fractionation used and the subgroup sizes it was not possible to assess the impact of the different RT modalities. Multivariable analyses identified completion of surgery, absence of symptoms at relapse, and female sex as prognostically favorable. Tumors with a 1q gain carried a higher cumulative incidence of dissemination after first relapse.Survival after recurrence was significantly influenced by symptoms and completeness of surgery. Only a homogeneous protocol with well posed, randomized questions could clarify the numerous issues, orient salvage treatment and ameliorate prognosis for this group of patients.
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000172531 650_7 $$2Other$$a1q gain
000172531 650_7 $$2Other$$acomplete surgery
000172531 650_7 $$2Other$$adissemination
000172531 650_7 $$2Other$$aependymoma relapse
000172531 650_7 $$2Other$$areirradiation
000172531 7001_ $$aBarretta, Francesco$$b1
000172531 7001_ $$aModena, Piergiorgio$$b2
000172531 7001_ $$0P:(DE-He78)3fdc3623477264cb5d0e14f256dbfbb8$$aJohann, Pascal$$b3$$udkfz
000172531 7001_ $$00000-0002-8744-8857$$aFerroli, Paolo$$b4
000172531 7001_ $$aAntonelli, Manila$$b5
000172531 7001_ $$aGandola, Lorenza$$b6
000172531 7001_ $$aGarrè, Maria Luisa$$b7
000172531 7001_ $$aBertin, Daniele$$b8
000172531 7001_ $$aMastronuzzi, Angela$$b9
000172531 7001_ $$aMascarin, Maurizio$$b10
000172531 7001_ $$aQuaglietta, Lucia$$b11
000172531 7001_ $$aViscardi, Elisabetta$$b12
000172531 7001_ $$aSardi, Iacopo$$b13
000172531 7001_ $$aRuggiero, Antonio$$b14
000172531 7001_ $$aBoschetti, Luna$$b15
000172531 7001_ $$aGiagnacovo, Marzia$$b16
000172531 7001_ $$aBiassoni, Veronica$$b17
000172531 7001_ $$aSchiavello, Elisabetta$$b18
000172531 7001_ $$aChiapparini, Luisa$$b19
000172531 7001_ $$aErbetta, Alessandra$$b20
000172531 7001_ $$aMussano, Anna$$b21
000172531 7001_ $$aGiussani, Carlo$$b22
000172531 7001_ $$aMura, Rosa Maria$$b23
000172531 7001_ $$aBarra, Salvina$$b24
000172531 7001_ $$aScarzello, Giovanni$$b25
000172531 7001_ $$aScimone, Giuseppe$$b26
000172531 7001_ $$00000-0002-5203-7855$$aCarai, Andrea$$b27
000172531 7001_ $$aGiangaspero, Felice$$b28
000172531 7001_ $$aButtarelli, Francesca Romana$$b29
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