Journal article
Pleomorphic xanthoastrocytoma with anaplasia and BEND5-NTRK2 fusion in a young adult with a history of cranial radiation for childhood rhabdomyosarcoma
Neuro-oncology advances, Vol.7(1), p.vdaf052
01/01/2025
DOI: 10.1093/noajnl/vdaf052
PMCID: PMC12188291
PMID: 40568680
Abstract
Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic tumor, with an anaplastic variant (PXA3) exhibiting more aggressive behavior and worse prognosis. We report the case of a young adult who developed PXA3 with a novel BEND5-NTRK2 fusion 2 decades after receiving cranial radiation for childhood rhabdomyosarcoma. The tumor's histopathology was challenging to classify, necessitating advanced molecular profiling, which confirmed the diagnosis. The identification of an NTRK2 fusion enabled targeted therapy with Larotrectinib, leading to sustained tumor control without further radiation. This case highlights the significance of comprehensive molecular testing in rare pediatric and young adult gliomas
Details
- Title: Subtitle
- Pleomorphic xanthoastrocytoma with anaplasia and BEND5-NTRK2 fusion in a young adult with a history of cranial radiation for childhood rhabdomyosarcoma
- Creators
- Julie M. Fischer - Univ Texas Hlth San Antonio, Dept Pediat Hematol & Oncol, San Antonio, TX 78229 USAAndrea R. Gilbert - The University of Texas Health Science Center at San AntonioEva M. Galvan - The University of Texas Health Science Center at San AntonioAchint K. Singh - The University of Texas Health Science Center at San AntonioJohn R. Floyd - The University of Texas Health Science Center at San AntonioShafqat Shah - The University of Texas Health Science Center at San Antonio
- Resource Type
- Journal article
- Publication Details
- Neuro-oncology advances, Vol.7(1), p.vdaf052
- DOI
- 10.1093/noajnl/vdaf052
- PMID
- 40568680
- PMCID
- PMC12188291
- NLM abbreviation
- Neurooncol Adv
- ISSN
- 2632-2498
- eISSN
- 2632-2498
- Publisher
- Oxford Univ Press
- Number of pages
- 5
- Language
- English
- Date published
- 01/01/2025
- Academic Unit
- Stead Family Department of Pediatrics; Hematology/Oncology
- Record Identifier
- 9984966847002771
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