Desmoplastic infantile ganglioglioma

Case contributed by Dr Sanjay Prabhu

Presentation

History of increasing irritability and head circumference. Outside hospital CT demonstrated a right supratentorial mass as well as hydrocephalus.

Patient Data

Age: 9 months
Gender: Male
MRI

MRI without and with contrast and MR Spectroscopy was performed

Large tumour that consists of both enhancing solid component and multiple cystic components with marked surrounding oedema, centered within the right frontal lobe. 

It causes subfalcine herniation to the left and obstructs the third ventricle at the level of foramen of Monro resulting in marked hydrocephalus Mass effect on mid-brain causing shift of mid-brain to the left and uncal herniation is noted. 

MR spectroscopy obtained from solid enhancing component of the lesion demonstrates markedly increased choline peak, consistent with high cellularity of this mass. NAA peak is not seen. The solid components of the tumor have restricted diffusion.

Differential diagnosis includes Primitive neuroectodermal tumor, atypical rhabdoid/ teratoid tumor, anaplastic ependymoma, extraventricular neurocytoma and desmoplastic infantile ganglioglioma.

Case Discussion

Desmoplastic infantile ganglioglioma:

  • composed of dural-based desmoplastic stromal tissue and neoplastic astrocytes
  • WHO Class 1
  • most patients present prior to 2 years of age
  • large cystic mass with enhancing nodules and adjacent pial/dural enhancement is characteristic
  • solid components are hypodense on T2-weighted images
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Case information

rID: 23298
Case created: 3rd Jun 2013
Last edited: 15th Sep 2015
System: Paediatrics
Inclusion in quiz mode: Included

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