Primary malignant ganglioglioma of the dorsolumbar spine (D11-L1).

We report an unusual patient with a primary malignant ganglioglioma, World Health Organization (WHO) grade III, of the spinal cord at the D11-L1 level in an 11-year-old boy. The patient presented with low backache and progressive paraparesis. A MRI scan revealed a well-defined conus lesion extending from D11 to L1, which was predominantly isointense on T1- and T2-weighted images and enhanced with contrast. On histopathological evaluation, the tumor was found to be comprised of two different components. One of the components revealed typical ganglioglioma with dysmorphic ganglion cells, positive for synaptophysin on immunohistochemistry. The other component was cellular with a high mitotic activity, giant tumor cells and glial fibrillary acidic protein (GFAP) positivity. The MIB-1 labeling index was ≥ 75% in the latter component. We emphasize the role of the MIB-1 labeling index to predict the prognosis and further management in these rare occurrences.