Faculty, Staff and Student Publications
Publication Date
5-9-2024
Journal
American Journal of Neuroradiology
DOI
10.3174/ajnr.A8192
PMID
38637027
PMCID
PMC11288540
PubMedCentral® Posted Date
5-1-2024
PubMedCentral® Full Text Version
Post-print
Abstract
Primary intracranial sarcoma, DICER1-mutant, is a rare, recently described entity in the fifth edition of the WHO Classification of CNS Tumors. Given the entity's rarity and recent description, imaging data on primary intracranial sarcoma, DICER1-mutant, remains scarce. In this multicenter case series, we present detailed multimodality imaging features of primary intracranial sarcoma, DICER1-mutant, with emphasis on the appearance of the entity on MR imaging. In total, 8 patients were included. In all 8 patients, the lesion demonstrated blood products on T1WI. In 7 patients, susceptibility-weighted imaging was obtained and demonstrated blood products. Primary intracranial sarcoma, DICER1-mutant, is a CNS neoplasm that primarily affects pediatric and young adult patients. In the present case series, we explore potential imaging findings that are helpful in suggesting this diagnosis. In younger patients, the presence of a cortical lesion with intralesional blood products on SWI and T1-weighted MR imaging, with or without extra-axial blood products, should prompt the inclusion of this entity in the differential diagnosis.
Keywords
Humans, Ribonuclease III, DEAD-box RNA Helicases, Male, Female, Brain Neoplasms, Mutation, Adolescent, Young Adult, Adult, Magnetic Resonance Imaging, Sarcoma, Child, Child, Preschool
Published Open-Access
yes
Recommended Citation
Eldaya, Rami W; Fagan, Richard J; Dagher, Samir A; et al., "Imaging Features of Primary Intracranial Sarcoma with DICER1 Mutation: A Multicenter Case Series" (2024). Faculty, Staff and Student Publications. 4393.
https://digitalcommons.library.tmc.edu/uthgsbs_docs/4393
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