Cover Image

Dramatic response to entrectinib in a rare glioneuronal tumor harboring an NTRK2 fusion

Show other versions (2)

Glioneuronal tumors are rare CNS neoplasms that can exhibit overlapping histological features with embryonal tumors, posing diagnostic and therapeutic challenges. We report a case of a 19-year-old Tunisian woman with a large right frontal tumor and contralateral extension. Initial partial resection...

Full description

Saved in:
Bibliographic Details
Main Authors: Akrout, Firas (Author) , Bogumil, Henri (Author) , Yedeas, Mohamed Dehmani (Author) , Sievers, Philipp (Author) , Touat, Mehdi (Author) , Achoura, Sameh (Author)
Format: Article (Journal)
Language:English
Published: January 2026
In: The oncologist
Year: 2026, Volume: 31, Issue: 1, Pages: 1-6
ISSN:1549-490X
DOI:10.1093/oncolo/oyaf418
Online Access:Verlag, kostenfrei, Volltext: https://doi.org/10.1093/oncolo/oyaf418
Get full text
Author Notes:Firas Akrout, Henri Bogumil, Mohamed Dehmani Yedeas, Philipp Sievers, Mehdi Touat, Sameh Achoura
Description
Summary:Glioneuronal tumors are rare CNS neoplasms that can exhibit overlapping histological features with embryonal tumors, posing diagnostic and therapeutic challenges. We report a case of a 19-year-old Tunisian woman with a large right frontal tumor and contralateral extension. Initial partial resection suggested CNS neuroblastoma, and the patient underwent chemoradiotherapy with temporary disease control. Upon progression, a second partial resection was followed by DNA methylation profiling, which reclassified the tumor as a glioneuronal tumor with ATRX alteration, kinase fusion, and anaplastic features (GTAKA), harboring a KANK1::NTRK2 fusion. Entrectinib therapy was initiated, leading to a complete radiological response at 14 months, with marked clinical improvement and no serious adverse effects. This case highlights the essential role of DNA methylation profiling in resolving diagnostic ambiguity and guiding targeted treatment in CNS tumors. It further supports the potential efficacy of entrectinib in NTRK fusion-positive glioneuronal tumors.
Item Description:Veröffentlicht: 15. Dezember 2025
Gesehen am 12.03.2026
Physical Description:Online Resource
ISSN:1549-490X
DOI:10.1093/oncolo/oyaf418

Similar Items