ArticleBrain pathology (Zurich, Switzerland)2018
Astroblastoma: a distinct tumor entity characterized by alterations of the X chromosome and MN1 rearrangement.
Article in Brain pathology (Zurich, Switzerland), 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers, 1 of them a synthesis that pooled it.
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Who cites it
39 citing papers in PubMed, 1 synthesis or guideline pooled it, 63 citations in OpenAlex.
- cIMPACT-NOW update 6: new entity and diagnostic principle recommendations of the cIMPACT-Utrecht meeting on future CNS tumor classification and grading.Brain pathology (Zurich, Switzerland) · 2020Guideline
- AOSNP-ADAPTR resource level-based recommendations on practical diagnostic strategies for ependymomas.Brain pathology (Zurich, Switzerland) · 2026Article
- Bridging pediatric and adult neuro-oncology: Insights into adolescents and young adults (AYA) central nervous system tumors.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2026Review
- Astroblastoma-like tumor with an EWSR1::BEND2 fusion in the fourth ventricle: a case report and narrative review.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Review
- Soft-tissue sarcoma with MN1-BEND2 fusion: second reported case and comparative analysis.Journal of surgical case reports · 2026Article
- A novel BEND2 monoclonal antibody enables highly specific immunohistochemical diagnosis of BEND2-fusion astroblastomas.Acta neuropathologica · 2026Article
- Molecular and clinical stratification of astroblastomas: Three distinct fusion-defined groups informing risk-adapted treatment strategies.Neuro-oncology · 2026Article
- Soft tissue sarcoma with MN1 gene fusions: a report of three cases with aggressive clinical behavior.The Journal of pathology · 2025Article
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- Posterior fossa astroblastoma: a case report of an extremely rare tumor with challenging diagnosis in a child and a review of literature.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Review
- MicroRNAs Expression Profile in MN1-Altered Astroblastoma.Biomedicines · 2025Article
- Clinicopathological and molecular characterization of astrocytoma.Frontiers in molecular neuroscience · 2025Article
- Molecular markers for pediatric low-grade glioma.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- Approaches to supratentorial brain tumours in children.Neuroradiology · 2024Review
- A case of disseminated spinal astroblastoma harboring a MAMLD1::BEND2 fusion.Neuropathology : official journal of the Japanese Society of Neuropathology · 2024Article
- Recent updates in pediatric diffuse glioma classification: insights and conclusions from the WHO 5Journal of medicine and life · 2024Review
- A novel FUS::BEND2 fusion expanding the molecular spectrum of astroblastomas.Free neuropathology · 2024Article
- Long-read sequencing for brain tumors.Frontiers in oncology · 2024Review
- Pediatric CNS tumors and 2021 WHO classification: what do oncologists need from pathologists?Frontiers in molecular neuroscience · 2024Review
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Authors and funding
10 authors at 8 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Astroblastoma is a rare, enigmatic tumor of the central nervous system (CNS) which shares some clinicopathologic aspects with other CNS tumors, especially ependymoma. To further clarify the nature of astroblastoma, we performed clinicopathologic and molecular genetic studies on eight cases of astroblastoma. The median age of the patients was 14.5 years, ranging from 5 to 60 years, and seven of the patients were female. All tumors arose in the cerebral hemisphere and radiologically appeared to be well-bordered, nodular tumors often associated with cystic areas and contrast-enhancement. Six of the seven patients with prognosis data survived without recurrences during the follow-up periods ranging from six to 76 months. One patient had multiple recurrences and died six years later. All tumors exhibited salient microscopic features, such as being well demarcated from the surrounding brain tissue, perivascular arrangement of epithelioid tumor cells (represented by "astroblastic" pseudorosettes, trabecular alignment, and pseudopapillary patterns), and hyalinized blood vessels. Immunoreactivity for GFAP, S-100 protein, Olig2, and EMA was variably demonstrated in all tumors, and IDH1 R132H and L1CAM were negative. Array comparative genomic hybridization revealed numerous heterozygous deletions on chromosome X in the four tumors studied, and break-apart fluorescence in situ hybridization demonstrated rearrangement of MN1 in five tumors with successful testing. The characteristic clinicopathologic and genetic findings support the idea that astroblastoma is distinct from other CNS tumors, in particular, ependymoma. In addition, MN1 rearrangement and aberrations of chromosome X may partly be involved in the pathogenesis of astroblastoma.
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