ArticleNeuro-oncology2022
Comprehensive profiling of myxopapillary ependymomas identifies a distinct molecular subtype with relapsing disease.
Article in Neuro-oncology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 32 citations in OpenAlex.
- AOSNP-ADAPTR resource level-based recommendations on practical diagnostic strategies for ependymomas.Brain pathology (Zurich, Switzerland) · 2026Article
- Spinal low-grade ependymal tumors harboring telomerase reverse transcriptase promoter mutation and chromosome 7 gain with methylation profile of spinal subependymoma.Acta neuropathologica communications · 2026Article
- A brief history of ependymoma.Neuro-oncology · 2026Review
- Single-nucleus transcriptomics of spinal ependymoma subtypes recognizes intratumoral heterogeneity.Neuro-oncology · 2026Article
- Multi-omics analysis delineates molecular signatures of spinal ependymal tumor.Cellular oncology (Dordrecht, Netherlands) · 2025Article
- Expansion of the spectrum of tumors diagnosed as myxopapillary ependymomas.Acta neuropathologica · 2025Article
- Strong nuclear expression of HOXB13 is a reliable surrogate marker for DNA methylome profiling to distinguish myxopapillary ependymoma from spinal ependymoma.Acta neuropathologica · 2025Article
- Central nervous system tumors in adolescents and young adults: A Society for Neuro-Oncology Consensus Review on diagnosis, management, and future directions.Neuro-oncology · 2025Review
- 40-Year-old man with two asynchronous spinal cord tumors.Brain pathology (Zurich, Switzerland) · 2024Article
- Specifics of spinal neuropathology in the molecular age.Neuro-oncology advances · 2024Review
- Spinal ependymal tumors.Neuro-oncology advances · 2024Review
- Integrated analyses reveal two molecularly and clinically distinct subtypes of H3 K27M-mutant diffuse midline gliomas with prognostic significance.Acta neuropathologica · 2024Article
- Morphology-based molecular classification of spinal cord ependymomas using deep neural networks.Brain pathology (Zurich, Switzerland) · 2024Article
- Outcomes and Pattern of Care for Spinal Myxopapillary Ependymoma in the Modern Era-A Population-Based Observational Study.Cancers · 2024Article
- Anaplastic histology and distinct molecular features in a small series of spinal cord ependymomas.Acta neuropathologica · 2024Article
- Glioma.Nature reviews. Disease primers · 2024Review
- [Explainable artificial intelligence in pathology].Pathologie (Heidelberg, Germany) · 2024Review
- Molecular characteristics and improved survival prediction in a cohort of 2023 ependymomas.Acta neuropathologica · 2024Article
- Transcriptomic and epigenetic dissection of spinal ependymoma (SP-EPN) identifies clinically relevant subtypes enriched for tumors with and without NF2 mutation.Acta neuropathologica · 2024Article
- Ependymoma from Benign to Highly Aggressive Diseases: A Review.Advances and technical standards in neurosurgery · 2024Review
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Authors and funding
28 authors at 10 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundMyxopapillary ependymoma (MPE) is a heterogeneous disease regarding histopathology and outcome. The underlying molecular biology is poorly understood, and markers that reliably predict the patients' clinical course are unknown.
methodsWe assembled a cohort of 185 tumors classified as MPE based on DNA methylation. Methylation patterns, copy number profiles, and MGMT promoter methylation were analyzed for all tumors, 106 tumors were evaluated histomorphologically, and RNA sequencing was performed for 37 cases. Based on methylation profiling, we defined two subtypes MPE-A and MPE-B, and explored associations with epidemiological, clinical, pathological, and molecular characteristics of these tumors.
resultsMPE-A occurred at a median age of 27 years and were enriched with tumors demonstrating papillary morphology and MGMT promoter hypermethylation. Half of these tumors could not be totally resected, and 85% relapsed within 10 years. Copy number alterations were more common in MPE-A. RNA sequencing revealed an enrichment for extracellular matrix and immune system-related signatures in MPE-A. MPE-B occurred at a median age of 45 years and included many tumors with a histological diagnosis of WHO grade II and tanycytic morphology. Patients within this subtype had a significantly better outcome with a relapse rate of 33% in 10 years (P = 3.4e-06).
conclusionsWe unraveled the morphological and clinical heterogeneity of MPE by identifying two molecularly distinct subtypes. These subtypes significantly differed in progression-free survival and will likely need different protocols for surveillance and treatment.
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