Ganglioglioma

Only one patient died 16 months after surgery due to an unrelated traffic accident.

P1, N=62, Active, not recruiting, Dana-Farber Cancer Institute | Recruiting --> Active, not recruiting | Trial completion date: Jan 2027 --> Jan 2028 | Trial primary completion date: Jan 2026 --> Jan 2027

BRAFV600E mutation was the most common alteration observed in gangliogliomas, whereas mutations in other genes, such as FGFR1, H3K27M, KRAS, IDH1, and RAF1, were rare.

P4, N=165, Active, not recruiting, Novartis Pharmaceuticals | Trial primary completion date: Jun 2026 --> Nov 2026

A second population of large round cells with abundant cytoplasm had positive cytoplasmic staining for S100 protein and synaptophysin (SYN), compatible with mature neurons. We diagnosed a spinal cord ganglioglioma in this steer based on histologic features and OLIG2 and SYN immunolabelling.

Thus, familiarity with relevant tumor mutations and radiogenomic features of LEATs is important for radiologists caring for affected patients. This article will review the genetic alterations and imaging characteristics of LEATs, formatted according to the three categories defined by the World Health Organization (WHO): glioneuronal and neuronal tumors (ganglioglioma, dysembryoplastic neuroepithelial tumor, papillary glioneuronal tumor, multinodular and vacuolating neuronal tumor); circumscribed astrocytic gliomas (pilocytic astrocytoma, pleomorphic xanthoastrocytoma); and pediatric-type diffuse low-grade gliomas (diffuse astrocytoma MYB or MYBL1-altered, angiocentric glioma, diffuse low-grade glioma MAPK pathway-altered, polymorphous low-grade neuroepithelial tumor of the young).

P4, N=165, Active, not recruiting, Novartis Pharmaceuticals | Recruiting --> Active, not recruiting

The most common molecular signature of these tumors is BRAF alterations, including rearrangements. The primary differential diagnosis is infant-type hemispheric glioma and given the similarities, pathologists must remain careful to ensure accurate diagnosis.

These findings highlight a previously unrecognized imaging pattern that may aid early identification of medullary GG and offer preliminary insight into its molecular basis. Further studies with larger cohorts are needed to validate these results.

P4, N=165, Recruiting, Novartis Pharmaceuticals | Active, not recruiting --> Recruiting | Trial completion date: Jul 2026 --> Nov 2026

In molecular level, PLNTY exhibits alterations in the MAPK pathway; while its DNA methylation profile demonstrates diversity. Most patients achieved seizure-free outcomes postoperatively, indicating a favorable prognosis.

H3K27M/BRAF V600E-mutant gliomas may represent a distinct subgroup, with outcomes linked to histologic and co-occurring molecular features. Targeted therapy and a surgical approach may support long-term survival in low-grade cases.