Evidence map›Paper›PMID 42399441›Full record

ReviewChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2026

Astroblastoma-like tumor with an EWSR1::BEND2 fusion in the fourth ventricle: a case report and narrative review.

Xiaoxia Yao, Libo Yang, Mengjia Shen, Yuyan Wei, Ni Chen, Zhang Zhang

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In one paragraph

Review in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

6 authors.

Xiaoxia YaoDepartment of Pathology, West China Hospital, Sichuan University, No.37, Guo Xue Xiang, Chengdu, 610041, Sichuan, China.
Libo YangDepartment of Pathology, West China Hospital, Sichuan University, No.37, Guo Xue Xiang, Chengdu, 610041, Sichuan, China.
Mengjia ShenDepartment of Pathology, West China Hospital, Sichuan University, No.37, Guo Xue Xiang, Chengdu, 610041, Sichuan, China.
Yuyan WeiDepartment of Pathology, West China Hospital, Sichuan University, No.37, Guo Xue Xiang, Chengdu, 610041, Sichuan, China.
Ni ChenDepartment of Pathology, West China Hospital, Sichuan University, No.37, Guo Xue Xiang, Chengdu, 610041, Sichuan, China.
Zhang ZhangDepartment of Pathology, West China Hospital, Sichuan University, No.37, Guo Xue Xiang, Chengdu, 610041, Sichuan, China. zhangzhang714@163.com.ORCID 0000-0002-4822-4471

Funding

National Natural Science Foundation of China 82401894Sichuan Provincial Science and Technology Support Program 2024YFFK0394
6 · The paper itself

Abstract

purposeTo report an EWSR1::BEND2 fusion astroblastoma-like tumor arising in the fourth ventricle of a 7-year-old girl and to highlight the diagnostic value of molecular pathology in astroblastoma-like tumors with an atypical immunophenotype.

methodsClinical, histopathological, immunohistochemical, and molecular findings of the present case are presented.

resultsDespite gross total resection followed by radiotherapy, the patient died 7 months after surgery. Histologically, the tumor showed typical astroblastoma-like morphology but an unusual immunophenotype characterized by complete loss of GFAP and Olig2 expression, focal EMA positivity, and diffuse SOX2 positivity. Because of this atypical immunoprofile, diagnosis was challenging and was ultimately established by molecular testing demonstrating an EWSR1::BEND2 fusion.

conclusionThis case highlights the critical role of molecular pathology in diagnosing astroblastoma-like tumors lacking conventional glial markers and suggests that SOX2 expression may represent a novel immunophenotypic finding in this tumor spectrum.

Indexed as

Cerebral Ventricle NeoplasmsFourth VentricleNeoplasms, NeuroepithelialRNA-Binding Protein EWSChildFatal OutcomeFemaleHumansEWSR1 protein, humanRNA-Binding Protein EWSAstroblastoma-like tumorEWSR1::BEND2 fusionFourth ventricleMolecular diagnosis

Identifiers

PMID42399441

What Socratic holds

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.