Desmoplastic infantile gangliogliomas are rare neoplasms, that have been considered distinct clinicopathologic entities. All reported cases are supratentorial, usually superficially situated with frequent extension to subarachnoid space and involvement of multiple lobes in more than half the cases. As in the present case, these tumors are characteristically firm due to extensive desmoplasia. Histologically tumor is composed of a mixture of spindle or enlarged astrocytes and occasional neuronal cells in a densely fibrous stroma. Immunohistochemistry demonstrates extensive glial differentiation but also neuronal epitopes in tumor cells with ambiguous features.
Cells often binucleate that morphologically ganglion cells are also present.
The cellular pleomorphism and atypia are frequently observed in this tumor and a diagnosis of a high-grade neoplasm should be avoided. If total surgical resection can be achieved, further therapy may not be indicated. In those patients with residual tumor, chemotherapy appears to be an effective form of therapy. Although DIGs are thought to be indolent tumors, subsequent follow up demonstrated continued growth of residual tumor encouraging re-operation. Tumor progression determined by image studies occurred as early as 2 months after combined surgical resection and chemotherapy.