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Article

Rare child primary intracranial sarcoma associated with DICER1 mutation: a case report and review of the literature.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery - 6 Jul 2026

Fan YaXuan, Zhang AXue, Wu Peng, Liu AnGen, Zhang Xing, Yin Li

Abstract excerpt

BACKGROUND: Primary intracranial sarcoma with DICER1 mutation (PIS-DICER1) is a rare and highly aggressive tumor that typically occurs in children and adolescents. Its clinical, radiologic, and pathological features are often nonspecific, making accurate diagnosis challenging. CASE PRESENTATION: We describe a 14-year-old boy who presented with dizziness and exertional intermittent headache. MRI revealed a...

Topics

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Adolescent
Brain Neoplasms
DEAD-box RNA Helicases
DICER1 gene
PDGFRB gene

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Ribonuclease III

Source

Open in PubMed Open full text

Identifiers

PMID: 42410089
DOI: 10.1007/s00381-026-07364-z
PMCID: PMC13337790

Genes

DICER1
PDGFRB
TP53
VIP

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026.

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