Clinical Heterogeneity of Desmoplastic Infantile Ganglioglioma

Desmoplastic infantile gangliogliomas (DIG) are intracranial tumors described in 1987 as benign lesions of infancy. A literature review and the clinical course of 3 patients reported herein suggest that the initial description should be amended. Nearly 23% of DIG cases occur in children older than 24 months. Approximately 40% of DIG cases require additional medical, radiation, and/or further surgical intervention, and 15% of infants and children develop leptomeningeal spread or die from DIG. Such adverse outcomes, combined with the recognition that DIG represents a heterogeneous disease, underscore the need for an expanded biological and molecular investigation.