Journal Article DKFZ-2020-02349

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The AIEOP 2nd series of children and adolescents intracranial Ependymoma. An integrated molecular and clinical characterization with a long-term follow-up.

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2021
Oxford Univ. Press Oxford

Neuro-Oncology 23(5), 848-857 () [10.1093/neuonc/noaa257]
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Abstract: 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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Note: 2021 May 5;23(5):848-857

Contributing Institute(s):
  1. B062 Pädiatrische Neuroonkologie (B062)
  2. DKTK HD zentral (HD01)
Research Program(s):
  1. 312 - Funktionelle und strukturelle Genomforschung (POF4-312) (POF4-312)

Appears in the scientific report 2021
Database coverage:
Medline ; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Essential Science Indicators ; IF >= 10 ; JCR ; NCBI Molecular Biology Database ; NationallizenzNationallizenz ; PubMed Central ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection
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 Record created 2020-11-04, last modified 2024-02-29



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