Peds (mainly) tumors - treatment

Question 1

EANO rec:
H3 K27M-mutant diffuse midline glioma, WHO grade 4

Answer 1

• Radiotherapy only.

This tumour type includes the majority of brainstem, thalamic and spinal diffuse gliomas in children and adults. Surgical options are limited and benefits from treatment options beyond radiotherapy have not been established because these tumours are rare and have not been studied in dedicated trials. In these tumours, the MGMT promoter is usually unmethylated. The prognosis of patients with this tumour type is poor.

Question 2

EANO rec:
H3.3 G34-mutant diffuse hemispheric glioma, WHO grade 4

Answer 2

• Surgery if feasible and Chemoradiotherapy.

These tumours mostly occur in adolescents and young adults and the MGMT promoter is more often methylated than unmethylated. These tumours were previously classified as IDH-wild-type glioblastomas and, thus, a reasonable treatment approach for such patients is chemoradiotherapy