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Imaging Features of Primary Intracranial Sarcoma with DICER1 Mutation: A Multicenter Case Series
Rami W Eldaya1, Richard J Fagan2, Samir A Dagher3
1From the Department of Neuroradiology (R.W.E., S.A.D., G.N.F., M.W.), The University of Texas MD Anderson Cancer Center, Houston, Texas reldaya@mdanderson.org.
AJNR. American Journal of Neuroradiology
|April 18, 2024
Summary
Newly identified DICER1-mutant primary intracranial sarcoma is rare. This study details its imaging features, noting blood products on MRI are key indicators in pediatric and young adult patients.
Area of Science:
- Neuropathology
- Neuroradiology
- Oncology
Background:
- Primary intracranial sarcoma, DICER1-mutant, is a recently classified CNS tumor.
- Limited imaging data exists for this rare entity.
- It primarily affects pediatric and young adult populations.
Purpose of the Study:
- To describe the multimodality imaging features of primary intracranial sarcoma, DICER1-mutant.
- To emphasize the MR imaging characteristics of this rare CNS neoplasm.
- To identify potential imaging findings aiding in diagnosis.
Main Methods:
- Multicenter case series.
- Inclusion of 8 patients diagnosed with primary intracranial sarcoma, DICER1-mutant.
- Detailed analysis of multimodality imaging, focusing on MR imaging sequences (T1WI, SWI).
Main Results:
- All 8 patients showed intralesional blood products on T1-weighted imaging (T1WI).
- 7 patients exhibited blood products on susceptibility-weighted imaging (SWI).
- Cortical location with intralesional/extra-axial blood products on SWI and T1WI was observed.
Conclusions:
- Imaging findings, particularly blood products on SWI and T1WI, can suggest DICER1-mutant primary intracranial sarcoma.
- This diagnosis should be considered in younger patients presenting with these specific imaging characteristics.
- Further research into rare CNS neoplasms like DICER1-mutant sarcoma is warranted.
