A rare case of supratentorial desmoplastic noninfantile ganglioglioma with heterogeneous differentiation and calcification

Abstract

Desmoplastic infantile gangliogliomas (DIGs) are rare supratentorial, neuroepithelial low-grade brain tumors that arise in the cerebral hemispheres and represent 1.25% of all intracranial tumors in children. They present commonly in the infantile age group within the first 2 years of life and are exceedingly rare in the noninfantile population. Less than sixty cases of this tumor type have been reported in literature. We present here a 9-year-old female child with a huge right frontoparietal DIG with midline shift and mass effect on ventricles. This rare case highlights the remarkable heterogeneous nature of this pediatric tumor and the importance of appropriate immunohistochemical examination for accurate pathologic diagnosis.