Desmoplastic infantile ganglioglioma

Desmoplastic infantile ganglioglioma (DIG) is a rare supratentorial neuroepithelial neoplasm Predominantly occurring in children under 1 year of age. In this report, the authors present the clinical course, radiographic findings, and histologic and ultrastructural characteristics of the tumor in a 6-month-old girl, who presented with intermittent seizure activity. Cranial computed tomography and magnetic resonance imaging revealed a large, dura-based cystic lesion with an enhancing solid component in the right parietal-occipital lobe. The tumor histologically was characterized by a leptomeningeal-based growth pattern with intense desmoplasia and divergent astroglial and ganglionic differentiation identified through immunohistochemistry and electron microscopy. Eleven months after complete surgical resection, the girl shows normal development without residual neurologic sequelae. Typically, the majority of these tumors are slow growing, appear histologically benign, and histogenetically thought to be hamartomas rather than true neoplasms. Complete surgical resection is considered curative.