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| Home > Publications database > The AIEOP 2nd series of children and adolescents intracranial Ependymoma. An integrated molecular and clinical characterization with a long-term follow-up. > print |
| Home > Publications database > The AIEOP 2nd series of children and adolescents intracranial Ependymoma. An integrated molecular and clinical characterization with a long-term follow-up. > print |
| 001 | 164263 | ||
| 005 | 20240229133513.0 | ||
| 024 | 7 | _ | |a 10.1093/neuonc/noaa257 |2 doi |
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| 082 | _ | _ | |a 610 |
| 100 | 1 | _ | |a Massimino, Maura |b 0 |
| 245 | _ | _ | |a The AIEOP 2nd series of children and adolescents intracranial Ependymoma. An integrated molecular and clinical characterization with a long-term follow-up. |
| 260 | _ | _ | |a Oxford |c 2021 |b Oxford Univ. Press |
| 336 | 7 | _ | |a article |2 DRIVER |
| 336 | 7 | _ | |a Output Types/Journal article |2 DataCite |
| 336 | 7 | _ | |a Journal Article |b journal |m journal |0 PUB:(DE-HGF)16 |s 1620733483_5168 |2 PUB:(DE-HGF) |
| 336 | 7 | _ | |a ARTICLE |2 BibTeX |
| 336 | 7 | _ | |a JOURNAL_ARTICLE |2 ORCID |
| 336 | 7 | _ | |a Journal Article |0 0 |2 EndNote |
| 500 | _ | _ | |a 2021 May 5;23(5):848-857 |
| 520 | _ | _ | |a A prospective 2002-2014 study stratified 160 patients by resection extent and histological grade, reporting results in 2016. We reanalyzed the series after a median 119 months, adding retrospectively patients' molecular features.Follow-up of all patients was updated. DNA copy-number analysis and gene-fusion detection could be completed for 94/160 patients, methylation classification for 68.PFS and OS at five/ten years were 66/58%, and 80/73%. Ten patients had late relapses (range 66-126 months), surviving after relapse no longer than those relapsing earlier (0-5 years). On multivariable analysis a better PFS was associated with grade 2 tumor and complete surgery at diagnosis and/or at RT; female sex and complete resection showed a positive association with OS. Posterior fossa(PF) tumors scoring ≥0.80 on DNA methylation analysis were classified as PFA (41) and PFB (8). PFB patients had better PFS and OS. Eighteen/32 supratentorial(ST) tumors were classified as RELA, and 3 as other molecular entities (anaplastic PXA, LGG MYB, HGNET). RELA had no prognostic impact. Patients with 1q gain or CDKN2A loss had worse outcomes, included significantly more patients >3 years old (p = 0.050) and cases of dissemination at relapse (p = 0.007).Previously-described prognostic factors were confirmed at 10-year follow-up. Late relapses occurred in 6.2% of patients. Specific molecular features may affect outcome: PFB patients had a very good prognosis; 1q gain and CDKN2A loss were associated with dissemination. To draw reliable conclusions, modern ependymoma trials need to combine diagnostics with molecular risk stratification and long-term follow-up. |
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| 700 | 1 | _ | |a Barretta, Francesco |b 1 |
| 700 | 1 | _ | |a Modena, Piergiorgio |b 2 |
| 700 | 1 | _ | |a Witt, Hendrik |0 P:(DE-He78)046fd145f1008f83f6236580727bbc0f |b 3 |u dkfz |
| 700 | 1 | _ | |a Minasi, Simone |b 4 |
| 700 | 1 | _ | |a Pfister, Stefan M |0 P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9 |b 5 |u dkfz |
| 700 | 1 | _ | |a Pajtler, Kristian W |0 P:(DE-He78)a7c1bbac024fa232d9c6b78443328d9d |b 6 |u dkfz |
| 700 | 1 | _ | |a Antonelli, Manila |b 7 |
| 700 | 1 | _ | |a Gandola, Lorenza |b 8 |
| 700 | 1 | _ | |a Garrè, Maria Luisa |b 9 |
| 700 | 1 | _ | |a Bertin, Daniele |b 10 |
| 700 | 1 | _ | |a Mastronuzzi, Angela |b 11 |
| 700 | 1 | _ | |a Mascarin, Maurizio |b 12 |
| 700 | 1 | _ | |a Quaglietta, Lucia |b 13 |
| 700 | 1 | _ | |a Viscardi, Elisabetta |b 14 |
| 700 | 1 | _ | |a Sardi, Iacopo |b 15 |
| 700 | 1 | _ | |a Ruggiero, Antonio |b 16 |
| 700 | 1 | _ | |a Pollo, Bianca |b 17 |
| 700 | 1 | _ | |a Buccoliero, Annamaria |b 18 |
| 700 | 1 | _ | |a Boschetti, Luna |b 19 |
| 700 | 1 | _ | |a Schiavello, Elisabetta |b 20 |
| 700 | 1 | _ | |a Chiapparini, Luisa |b 21 |
| 700 | 1 | _ | |a Erbetta, Alessandra |b 22 |
| 700 | 1 | _ | |a Morra, Isabella |b 23 |
| 700 | 1 | _ | |a Gessi, Marco |b 24 |
| 700 | 1 | _ | |a Donofrio, Vittoria |b 25 |
| 700 | 1 | _ | |a Patriarca, Carlo |b 26 |
| 700 | 1 | _ | |a Giangaspero, Felice |b 27 |
| 700 | 1 | _ | |a Johann, Pascal |0 P:(DE-He78)3fdc3623477264cb5d0e14f256dbfbb8 |b 28 |u dkfz |
| 700 | 1 | _ | |a Buttarelli, Francesca Romana |b 29 |
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