Desmoplastic infantile ganglioglioma

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 tumor of infancy characterized 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 tumors 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.

    Create a new playlist
Loading...