Desmoplastic infantile ganglioglioma (DIG) is an uncommon neuroepithelial tumor associated with epilepsy, mostly occurring in the first 2 years of life. Most DIGs carry good prognosis after complete resection, even when a primitive cellular element is present. However, a few examples of DIG with histologic anaplasia have recently been reported, and one demonstrated an unusual aggressive behavior. The authors describe herein a DIG with high Ki-67 proliferation index (30%) in a 10-month-old male infant with epilepsy, but with an excellent prognosis after total tumor resection.

Keywords: Desmoplastic infantile ganglioglioma, Anaplastic features, Epilepsy