Evidence map›Paper›PMID 41995891›Full record

ArticleJournal of neuro-oncology2026

Low-grade neuroepithelial tumor with FGFR1::TACC1 fusion: a clinicopathologic analysis of seven cases.

Mei Yang, Danmei Zhou, Jie Fan, Xueling Qi, Jing Feng, Qiong Li, Yin Wang, Feng Tang, Ji Xiong, Zunguo Du

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Article in Journal of neuro-oncology, 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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5 · Who and what money

Authors and funding

10 authors.

Mei YangDepartment of Pathology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
Danmei ZhouDepartment of Pathology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
Jie FanDepartment of Pathology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
Xueling QiDepartment of Pathology, Sanbo Brain Hospital, Capital Medical University, Beijing, 100093, China.
Jing FengDepartment of Pathology, Sanbo Brain Hospital, Capital Medical University, Beijing, 100093, China.
Qiong LiDepartment of Pathology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
Yin WangDepartment of Pathology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
Feng TangDepartment of Pathology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
Ji XiongDepartment of Pathology, Huashan Hospital, Fudan University, Shanghai, 200040, China.
Zunguo DuDepartment of Pathology, Huashan Hospital, Fudan University, Shanghai, 200040, China. duzunguo@fudan.edu.cn.

Funding

Natural Science Foundation of Shanghai 25ZR1401037Shanghai Action Plan for Science Technology and Innovation 2023DFYCQN02
6 · The paper itself

Abstract

purposeTo characterize the clinicopathologic, radiologic, immunophenotypic, and molecular features of low-grade neuroepithelial tumor harboring FGFR1::TACC1 fusion.

methodsSeven cases with confirmed FGFR1::TACC1 fusion were retrospectively analyzed. Clinical information, histopathological features, immunohistochemical profiles, next-generation sequencing (NGS) results and DNA methylation–based clustering analyses were reviewed.

resultsAll patients were male, with a median age of 14 years. Tumors were predominantly located in the cerebellum. Magnetic resonance imaging revealed mainly cystic–solid lesions with hypointensity on T1-weighted imaging and relatively well-defined margins on FLAIR sequences. Histologically, all tumors exhibited oligodendroglia-like cells with round nuclei and perinuclear halos, accompanied by neurofibrillary islands and edematous stroma. Branching capillaries (6/7), calcifications (4/7), and Rosenthal fibers (3/7) were variably observed. One case demonstrated morphologic and immunophenotypic features overlapping with extraventricular neurocytoma. Immunohistochemistry demonstrated GFAP and Olig2 expression in all cases; Syn positivity was identified in four cases and focal NeuN positivity in two cases. Focal CD34 expression was found in one. The Ki-67 was below 5% in all tumors. NGS confirmed FGFR1::TACC1 fusion in every case. DNA methylation profiling assigned tumors to multiple reference classes, including low-grade glioma (LGG), dysembryoplastic neuroepithelial tumor (DNT), pilocytic astrocytoma, posterior fossa (PA PF) and anaplastic pilocytic astrocytoma (ANA PA). No significant chromosomal arm-level copy number alterations were detected. All patients underwent gross resection, with one receiving adjuvant radiotherapy. No tumor recurrence was observed during follow-up (36–60 months).

conclusionLow-grade neuroepithelial tumors with FGFR1::TACC1 fusion represent a heterogeneous entity where a single recurrent gene fusion may be associated with diverse histomorphologic features and variable DNA methylation profiles.

Indexed as

Brain NeoplasmsMicrotubule-Associated ProteinsNeoplasms, NeuroepithelialOncogene Proteins, FusionReceptor, Fibroblast Growth Factor, Type 1AdolescentAdultBiomarkers, TumorChildChild, PreschoolDNA MethylationFetal ProteinsHumansMaleNuclear ProteinsRetrospective StudiesBiomarkers, TumorFetal ProteinsFGFR1 protein, humanMicrotubule-Associated ProteinsNuclear ProteinsOncogene Proteins, FusionReceptor, Fibroblast Growth Factor, Type 1TACC1 protein, humanFGFR1Low-grade neuroepithelial tumorMethylation clusteringTACC1

Identifiers

PMID41995891

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