Related Experiment Video
Updated: Jun 28, 2025
Isolation and Characterization of Tumor-initiating Cells from Sarcoma Patient-derived Xenografts
Published on: June 13, 2019
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.
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.
Insights
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.
