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| Home > Publications Database > Therapeutic implications of improved molecular diagnostics for rare CNS embryonal tumor entities: results of an international, retrospective study. |
| Journal Article | DZNE-2021-01455 |
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2021
Oxford Univ. Press
Oxford
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Please use a persistent id in citations: doi:10.1093/neuonc/noab136
Abstract: Only few data are available on treatment-associated behavior of distinct rare CNS embryonal tumor entities previously treated as 'CNS-primitive neuroectodermal tumors' (CNS-PNET). Respective data on specific entities, including CNS neuroblastoma, FOXR2 activated (CNS NB-FOXR2), and embryonal tumors with multilayered rosettes (ETMR) are needed for development of differentiated treatment strategies.Within this retrospective, international study, tumor samples of clinically well-annotated patients with the original diagnosis of CNS-PNET were analyzed using DNA methylation arrays (n = 307). Additional cases (n = 66) with DNA methylation pattern of CNS NB-FOXR2 were included irrespective of initial histological diagnosis. Pooled clinical data (n = 292) were descriptively analyzed.DNA methylation profiling of 'CNS-PNET' classified 58 (19%) cases as ETMR, 57 (19%) as high-grade glioma (HGG), 36 (12%) as CNS NB-FOXR2, and 89(29%) cases were classified into 18 other entities. Sixty-seven (22%) cases did not show DNA methylation patterns similar to established CNS tumor reference classes. Best treatment results were achieved for CNS NB-FOXR2 patients (5-year PFS: 63% ± 7%, OS: 85% ± 5%, n = 63), with 35/42 progression-free survivors after upfront craniospinal irradiation (CSI) and chemotherapy. The worst outcome was seen for ETMR and HGG patients with 5-year PFS of 18% ± 6% and 22% ± 7%, and 5-year OS of 24% ± 6% and 25% ± 7%, respectively.The historically reported poor outcome of CNS-PNET patients becomes highly variable when tumors are molecularly classified based on DNA methylation profiling. Patients with CNS NB-FOXR2 responded well to current treatments and a standard-risk CSI-based regimen may be prospectively evaluated. The poor outcome of ETMR across applied treatment strategies substantiates the necessity for evaluation of novel treatments.
Keyword(s): Brain Neoplasms: diagnosis (MeSH) ; Brain Neoplasms: genetics (MeSH) ; Brain Neoplasms: therapy (MeSH) ; Central Nervous System Neoplasms: diagnosis (MeSH) ; Central Nervous System Neoplasms: genetics (MeSH) ; Central Nervous System Neoplasms: therapy (MeSH) ; Forkhead Transcription Factors (MeSH) ; Humans (MeSH) ; Neoplasms, Germ Cell and Embryonal: diagnosis (MeSH) ; Neoplasms, Germ Cell and Embryonal: genetics (MeSH) ; Neoplasms, Germ Cell and Embryonal: therapy (MeSH) ; Neuroectodermal Tumors, Primitive: diagnosis (MeSH) ; Neuroectodermal Tumors, Primitive: genetics (MeSH) ; Neuroectodermal Tumors, Primitive: therapy (MeSH) ; Pathology, Molecular (MeSH) ; Retrospective Studies (MeSH) ; CNS NB-FOXR2 ; CNS embryonal tumor ; CNS-PNET ; DNA methylation profiling ; ETMR ; FOXR2 protein, human ; Forkhead Transcription Factors
; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Essential Science Indicators ; IF >= 10 ; JCR ; Nationallizenz
; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection
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