TABLE 1.
Age at Diagnosis | Sex | Presenting symptoms | Tumor Location | Surgery* | Tumor Ki‐67 proliferative index | Tumor Mitoses | Histopathologic Diagnosis* | v12.5 Methylation Class (Score) | Fusion | Additional tumor cytogenetic and molecular features | Recurrence/progression | Follow‐up (months) | |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Case 1 | 8 years | Female | Seizure | Left frontal cortex | GTR | elevated (5‐10%) | Rare | LGG/LGNT, NOS | Pilocytic astrocytoma, hemispheric ( 0.6) | TRAK1::RAF1 | broad loss of Chromosome 3p | focal recurrent tumor noted on 14 month surveillance MRI; treated by complete re‐resection (NED at 5 month follow‐up) | 19 |
Case 2 | 9 months | Female | Decreased motor activity (arm/leg) | Left fronto‐parietal leptomeninges | GTR | low (< 5%) | Absent | DIG | Desmoplastic infantile ganglioglioma / desmoplastic infantile astrocytoma (1.0 ) | TRAK1::RAF1 | (none detected; germline Chr 15p deletion) | none | 53 |
Case 3 | 15 months | Female | Seizure | Right parietal/occipital leptomeninges | STR | elevated (>10%) | Rare | DLGNT | Desmoplastic infantile ganglioglioma / desmoplastic infantile astrocytoma (1.0 ) | TRAK1::RAF1 | (none detected) | none | 15 |
*Abbreviations: DLGNT = diffuse leptomeningeal glioneuronal tumor; GTR = gross total resection; LGG‐LGNT, NOS = low grade glial‐glioneuronal tumor, not other specified; NED = no evidence of disease; STR+ subtotal resection.