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000279157 005__ 20250713001400.0
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000279157 1001_ $$aKrech, Maja$$b0
000279157 245__ $$aOutcome-associated factors in a molecularly defined cohort of central neurocytoma.
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000279157 520__ $$aCentral neurocytomas (CN) are intraventricular brain tumors predominantly occurring in young adults. Although prognosis is usually favorable, tumor recurrence is common, particularly following subtotal resection (STR). Currently, the risk of progression is evaluated using atypical features and an elevated Ki67 proliferation index. However, these markers lack consistent definitions, raising the need for objective criteria. Genome-wide DNA methylation profiles were examined in 136 tumors histologically classified as CN. Clinical/histopathological characteristics were assessed in 93/90 cases, and whole-exome sequencing was conducted in 12 cases. Clinical and molecular characteristics were integrated into a survival model to predict progression-free survival (PFS). A diagnosis of CN was epigenetically confirmed in 125 of 136 cases (92%). No DNA methylation subgroups were identified, but global DNA hypomethylation emerged as a hallmark feature of CN associated with higher recurrence risk. Risk stratification based on histological features of atypia and Ki67 proliferation index was not reproducible across neuropathologists. Hypomethylation at the FGFR3 locus, accompanied by increased FGFR3 protein expression, was observed in 97% of cases. Gross total resection was associated with significantly improved PFS compared to STR, while patients undergoing STR receiving radiotherapy had a better outcome (p = 0.0001). Younger patients were identified as having a higher risk of recurrence (p = 0.026). Patient age and treatment strategy were key factors associated with survival outcomes in this cohort. These findings underscore the importance of closer follow-up for younger patients and radiotherapy for STR cases. Furthermore, FGFR3 represents a hallmark feature and potential therapeutic target, warranting further investigation.
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000279157 650_7 $$2Other$$aDNA methylation profiling
000279157 650_7 $$2Other$$aFGFR3
000279157 650_7 $$2Other$$aNeurocytoma
000279157 650_7 $$2Other$$aProgression-free survival
000279157 650_7 $$2Other$$aRadiotherapy
000279157 650_2 $$2MeSH$$aHumans
000279157 650_2 $$2MeSH$$aMale
000279157 650_2 $$2MeSH$$aFemale
000279157 650_2 $$2MeSH$$aNeurocytoma: genetics
000279157 650_2 $$2MeSH$$aNeurocytoma: pathology
000279157 650_2 $$2MeSH$$aAdult
000279157 650_2 $$2MeSH$$aYoung Adult
000279157 650_2 $$2MeSH$$aBrain Neoplasms: genetics
000279157 650_2 $$2MeSH$$aBrain Neoplasms: pathology
000279157 650_2 $$2MeSH$$aDNA Methylation
000279157 650_2 $$2MeSH$$aMiddle Aged
000279157 650_2 $$2MeSH$$aAdolescent
000279157 650_2 $$2MeSH$$aCohort Studies
000279157 650_2 $$2MeSH$$aNeoplasm Recurrence, Local: genetics
000279157 650_2 $$2MeSH$$aPrognosis
000279157 7001_ $$aMuench, Amos$$b1
000279157 7001_ $$aTeichmann, Daniel$$b2
000279157 7001_ $$aKuzman, Peter$$b3
000279157 7001_ $$aSuwala, Abigail K$$b4
000279157 7001_ $$aIppen, Franziska M$$b5
000279157 7001_ $$aMüther, Michael$$b6
000279157 7001_ $$aWeber, Katharina J$$b7
000279157 7001_ $$aWenger-Alakmeh, Katharina$$b8
000279157 7001_ $$aOnken, Julia$$b9
000279157 7001_ $$aVajkoczy, Peter$$b10
000279157 7001_ $$aBehling, Felix$$b11
000279157 7001_ $$aMay, Sven-Axel$$b12
000279157 7001_ $$aNtoulias, Georgios$$b13
000279157 7001_ $$aKrauss, Joachim K$$b14
000279157 7001_ $$aAtallah, Oday$$b15
000279157 7001_ $$aEsmaeilzadeh, Majid$$b16
000279157 7001_ $$aMueller, Wolf C$$b17
000279157 7001_ $$0P:(DE-2719)2812386$$aHeppner, Frank L$$b18$$udzne
000279157 7001_ $$aRadbruch, Helena$$b19
000279157 7001_ $$aDittmayer, Carsten$$b20
000279157 7001_ $$aStenzel, Werner$$b21
000279157 7001_ $$aKoch, Arend$$b22
000279157 7001_ $$aCapper, David$$b23
000279157 7001_ $$aKaul, David$$b24
000279157 7001_ $$aPaulus, Werner$$b25
000279157 7001_ $$aPlate, Karl H$$b26
000279157 7001_ $$aSteinbach, Joachim P$$b27
000279157 7001_ $$aCzabanka, Markus$$b28
000279157 7001_ $$aBeschorner, Rudi$$b29
000279157 7001_ $$avon Deimling, Andreas$$b30
000279157 7001_ $$aBockmayr, Michael$$b31
000279157 7001_ $$aNeumann, Julia E$$b32
000279157 7001_ $$aBrandner, Sebastian$$b33
000279157 7001_ $$aKrieger, Teresa$$b34
000279157 7001_ $$aHartmann, Christian$$b35
000279157 7001_ $$aThomas, Christian$$b36
000279157 7001_ $$aSchweizer, Leonille$$b37
000279157 773__ $$0PERI:(DE-600)1458410-4$$a10.1007/s00401-025-02894-3$$gVol. 149, no. 1, p. 61$$n1$$p61$$tActa neuropathologica$$v149$$x0001-6322$$y2025
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