HG-111: EARLY RECURRENCE OF GANGLIOGLIOMA IN A SURVIVOR OF INFANTILE GLIOBLASTOMA

INTRODUCTION: Infantile glioblastoma (GBM) is a rare tumor with a variable prognosis and clinical course. Despite harboring histologic features similar to older patients, these children may have improved survival and respond more favorably to surgery and chemotherapy. To highlight this tumor's behavior, we report the case of a long-term survivor of infantile GBM who developed recurrent tumor, later diagnosed as a ganglioglioma, in the surgical bed 6 months after gross total resection of her GBM. CASE: A 5 month old female presented with irritability, emesis, and increasing head circumference. Imaging demonstrated a 9cm parieto-occipital mass which was gross totally resected. The entirety of the tumor showed glioblastoma. She received adjuvant temozolomide and lomustine for 6 months, at which time a 1.3cm nodule along the resection cavity developed, consistent with recurrent disease. She was enrolled on a phase II study of the antiangiogenic drug cilengitide and received 20 months of treatment. Her tumor subsequently exhibited slow growth until it was resected. Histologic evaluation revealed a ganglioglioma. Karyotype was 46,XX for both tumors. No p53 abnormality was identified in the primary tumor. She has no evidence of disease, now 8 years from her initial diagnosis and 2 years from resection of ganglioglioma. CONCLUSION: This is the first known report of a recurrent ganglioglioma after a diagnosis of infantile GBM. Whether this represents tumor differentiation as a result of chemotherapy, or this tumor's particular natural history, is uncertain. This case further illustrates the unique biological and clinical characteristics of infantile GBM.