Primary intracranial sarcoma, DICER1-mutant

Mesenchymal, non-meningothelial tumors

General description

Primary intracranial sarcoma, DICER1-mutant represents a rare but increasingly recognized entity in the fifth edition of the WHO Classification of CNS Tumors. The epidemiological profile demonstrates a strong predilection for pediatric and young adult populations, with the median age at diagnosis ranging from 6 to 20 years.

References

  1. Eldaya, Rami W., et al. "Imaging Features of Primary Intracranial Sarcoma with DICER1 Mutation: A Multicenter Case Series." American Journal of Neuroradiology 45.5 (2024): 626-631.
  2. Marinelli, Alfredo, et al. "A rare adult primary intracranial sarcoma, DICER1-mutant identified by epigenomic profiling: A case report." Brain Sciences 13.2 (2023): 235.

Cerebral mass

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