Desmoplastic infantile ganglioglioma

Case contributed by Dr Odina Gomez

Presentation

Vomiting for 5 days.

Patient Data

Age: 5 months
Gender: Female

MRI Brain

Modality: MRI

Large, mixed signal intensity mass that is predominantly multicystic, multiseptate with heterogeneously enhancing solid lobulated component in the right middle cranial fossa centered in the right temporal lobe.  It exhibits significant mass effect effacing the right frontal and parietal lobes, and causing leftward subfalcine and downward transtentorial herniation.  There is compression of the right lateral ventricle, and mild dilatation of the left lateral ventricle.  

 

Brain ultrasound

Modality: Ultrasound

Large, multiseptate cystic mass located predominantly in the right frontal and temporal lobes, causing right lateral ventricular compression and subfalcine herniation

Case Discussion

Post temporal craniotomy with subtotal excision of tumor was done and biopsy revealed a mature glial-neuronal tumor with collagenous matrix, marked desmoplasia, and proliferative primitive component for which the diagnosis of Desmoplastic infantile ganglioglioma (WHO Grade 1) was made. 

Desmoplastic infantile ganglioglioma is a rare supratentorial tumour of infancy characterised by its massive size with cystic and enhancing solid components, having a predilection to at least two hemispheric lobes particularly frontal and parietal.  The median age at diagnosis was more presumptuous at around 5 or 6 months as in this patient who initially presented with vomiting. Despite their aggressive radiologic features, such tumours were noted to have a good prognosis after surgery.

The radiologic differential diagnoses of desmoplastic infantile tumors include primitive neuroectodermal tumor (PNET), dysembryoplastic neuroepithelial tumor (DNET), ependymoma, and ganglioglioma.

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Case Information

rID: 40211
Case created: 12th Oct 2015
Last edited: 25th Oct 2015
Inclusion in quiz mode: Included

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