Central nervous system schwannoma, VGLL-fused (EWSR1::VGLL1 fusion) with neuroblastoma-like cell dense areas in the frontal lobe of a young man with schwannomatosis due to a germline LZTR1 mutation

We report a central nervous system schwannoma, <i>VGLL</i>-fused in a young man's frontal lobe. Somatic abnormalities included an <i>EWSR1::VGLL1</i> fusion, which incorporated the entire translated region of <i>VGLL1</i>, but excluded most domains of <i>EWSR1</i>. The tumor histologically merged with the brain, and showed both schwannoma-like and neuroblastoma-like areas. A germline <i>LZTR1</i> mutation was subsequently identified, implying the patient suffered from schwannomatosis.