Desmoplastic non-infantile ganglioglioma

Abstract

Desmoplastic ganglioglioma is a rare, markedly desmoplastic variant of ganglioglioma that usually presents in the first year of life. It is a mixed glial and neuronal cerebral tumor. A few cases of desmoplastic ganglioglioma have been reported in non-infantile patients. We report a case of desmoplastic ganglioglioma in a 14-year-old boy. The patient presented with a large solid cystic mass with mild peritumoral edema in the right posterior parieto-occipital region with direct contact to the falx cerebri. Histopathological examination revealed a low-grade glial tumor with prominent desmoplasia including hypocellular collagenous areas. The tumor was well demarcated with respect to the surrounding brain. Ganglion cells with dysplastic features were present in the tumor and clustered in some areas. Focal lymphocytic infiltration was also observed. Mitotic activity was very low. Immunohistochemistry revealed glial fibrillary acidic protein positivity in the astrocytic cells hidden in the desmoplastic tissue. Synaptophysin and neuron specific enolase were positive in ganglion-like neuronal cells. The MIB-1 labeling index was less than 1%. This present case confirms that desmoplastic ganglioglioma can be present in young adult patients with its characteristic radiologic features.