Neurosurgery notes/Tumours/Gliomas, Glioneuronal Tumors, and Neuronal Tumors/Paediatrics type diffuse astrocytoma/Paediatric-type high-grade diffuse gliomas/Infant-type hemispheric glioma

Infant-type hemispheric glioma

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Paediatric-type high-grade diffuse gliomas

Definition

  • Essential:
    • Cellular astrocytoma AND
    • Presentation in early childhood AND
    • Cerebral hemispheric location AND
      • Presence of a typical receptor tyrosine kinase abnormality (e.g. fusion in an NTRK family gene or in ROS1, MET1, or ALK) OR
      • Methylation profile aligned with infant-type hemispheric glioma

Subtypes

  • Base on receptor tyrosine kinase fusions
    • ALK-altered
    • ROS1-altered
    • NTRK1/2/3-altered
    • ME-altered

Numbers

  • All reported cases have been in early childhood with a median age at presentation of 2.8 months

Clinical presentation

  • Non-specific symptoms such as
    • Lethargy
    • Increased head circumference.

Location

  • These tumours occur supratentorially, usually as large masses that can demonstrate superficial involvement including leptomeningeal extension.

Radiology

  • Tumours presented with solid and large internal cystic components and areas of intratumoural haemorrhage
  • The total number of cases within each molecular subtype (NTRK-altered, ROS1-altered, ALK-altered, and MET-altered) remains extremely small precluding further description here.
  • Spinal MRI is recommended
    • Occasional cases have shown leptomeningeal dissemination;
  • T2w (A), T1w-CE (B, C), and ADC (D) show
    • A large right-sided MCA and ACA territory infarct.
    • The right frontal lobe solid and cystic tumour is hyperintense on T2w.
    • Assessment of diffusion characteristics on ADC (D) is hampered by the presence of haemorrhage SWI (E) in the tumour and ventricular system, but diffusion of solid tumour components is facilitated.
    • The tumour infiltrates the adjacent frontal horn.
    • There is intraspinal leptomeningeal disease (arrows in C)
A close-up of a brain scan AI-generated content may be incorrect.
A 14-week-old boy presented with symptoms of sepsis, seizures, poor feeding, and vomiting. Fusion panel sequencing: TRIM24-MET fusion.

Histopathology

Microscopic

  • Predominantly astrocytic cytology, with spindled nuclei, gemistocytic‑like cells, and in some cases a focal ganglion‑cell component.
  • Architecture is usually of uniform sheets of tumor cells, although some cases show a more nodular pattern; occasional cases are less densely cellular.
 
notion image

Outcome

  • Better survival than adult variants of high grade gliomas.