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Status |
Public on Oct 23, 2024 |
Title |
Impact of molecular classification on prognosis in children and adolescents with spinal ependymoma: Results from the HIT-MED database |
Organism |
Homo sapiens |
Experiment type |
Third-party reanalysis Methylation profiling by array
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Summary |
Ependymomas of the spinal cord are rare among children and adolescents, and the individual risk of disease progression is difficult to predict. This study aims at evaluating the prognostic impact of molecular typing of pediatric spinal cord ependymomas. Eighty-three patients with spinal ependymomas ≦ 22 years registered in the HIT-MED database between 1992 and 2022 were included. Forty-seven tumors were analyzed by DNA methylation array profiling. In six cases, HOXB13 and MYCN proteins were detected as surrogate markers for specific methylation classes. With a median follow-up time of 4.9 years (y), 5y- and 10y-overall survival (OS) were 100% and 86%, while 5y- and 10y-progression-free survival (PFS) were 65% and 54%. Myxopapillary ependymoma (SP-MPE, n=32, 63%) was the most common molecular type followed by spinal ependymoma (SP-EPN, n=17, 33%) and MYCN-amplified ependymoma (n=2, 4%). One case could not be molecularly classified, and one was reclassified as anaplastic pilocytic astrocytoma. 5y-PFS did not significantly differ between SP-MPE and SP-EPN (65% versus 78%, p=0.64). MYCN-amplification was associated with early relapses (<2.3y) in both cases and death in one patient. Patients with SP-MPE subtype B (n=9) showed a non-significant trend for better 5y-PFS compared to subtype A (n=18; 86% versus 56%, p=0.15). The extent of resection and WHO tumor grades significantly influenced PFS in a uni- and multivariate analysis.
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Overall design |
47 specicmens were analyzed using the Illumina Infinium HumanMethylation450 or the MethylationEPIC BeadChip.
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Web link |
https://academic.oup.com/noa/article/6/1/vdae179/7832557
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Contributor(s) |
Engertsberger L, Schüller U |
Citation(s) |
- Engertsberger L, Benesch M, Mynarek M, Tonn S et al. Impact of molecular classification on prognosis in children and adolescents with spinal ependymoma: Results from the HIT-MED database. Neurooncol Adv 2024 Jan-Dec;6(1):vdae179. PMID: 39713042
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Submission date |
Sep 11, 2024 |
Last update date |
Jan 22, 2025 |
Contact name |
Lara Engertsberger |
E-mail(s) |
lara.engertsberger@medunigraz.at
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Organization name |
Medical University of Graz
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Department |
Division of Pediatric Hemato-Oncology
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Street address |
Auenbruggerplatz 34/2
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City |
Graz |
ZIP/Postal code |
8036 |
Country |
Austria |
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Platforms (2) |
GPL13534 |
Illumina HumanMethylation450 BeadChip (HumanMethylation450_15017482) |
GPL21145 |
Infinium MethylationEPIC |
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