Primary CNS sarcoma, DICER1-mutant with rhabdomyoblastic differentiation arising within the thoracic spine: illustrative case

Background: Primary CNS sarcoma, DICER1-mutant is almost exclusively found in the supratentorial compartment. Isolated spinal lesions are exceedingly rare.

Observations: The authors present a case of thoracic intradural extramedullary primary CNS sarcoma, DICER1-mutant. An adult male with a remote history of a left cerebellar lesion resection with unknown pathology presented with sensation changes extending caudally from the torso. He subsequently underwent laminectomy and tumor resection of the lesion. The patient experienced recurrence at 1.5 months requiring re-resection for palliative decompression.