Clinicohistoradiological and surgical outcome in diffuse midline glioma

Childs Nerv Syst. 2024 Jan;40(1):65-71. doi: 10.1007/s00381-023-06095-9. Epub 2023 Aug 30.

Abstract

Purpose: Diffuse Midline Glioma (DMG) with H3K27M mutation is a rare and aggressive midline high grade glioma with a predominant astrocytic differentiation and K27M mutation in either H3F3A or HIST1H3B/C. This tumor is more common in children than in adults. The current study was aimed to determine clinicohistoradiological and surgical outcome of patients who have undergone surgery for DMG and study disease severity of patients with DMG.

Methods: This is an observational study in which 29 DMG patients were evaluated for clinicohistoradiological and surgical outcomes by assessing the pre and postoperative neurological status.

Result: Survival duration was significantly high in patients with age > 18 years (p = 0.02). Patients who had undergone Radiation Therapy showed higher survival rate (p = 0.05) and the cases with low levels of Ki 67 index had improved post operative outcome (p = 0.002).

Conclusion: DMG with H3K27M mutation in newly classified Central Nervous System tumor are WHO grade IV Tumors, comprising H3K27M mutation as molecular marker for diagnosis and related with a poor prognosis.

Keywords: Brain tumor; Diffuse Midline Glioma (DMG); H3K27M mutation; Ki 67 index; Pediatric tumor.

Publication types

  • Observational Study

MeSH terms

  • Adult
  • Brain Neoplasms* / genetics
  • Brain Neoplasms* / pathology
  • Brain Neoplasms* / surgery
  • Child
  • Glioma* / diagnosis
  • Glioma* / genetics
  • Glioma* / surgery
  • Histones / genetics
  • Humans
  • Middle Aged
  • Mutation / genetics
  • Treatment Outcome

Substances

  • Histones