Desmoplastic noninfantile ganglioglioma: report of a case

Desmoplastic infantile ganglioglioma is a rare superficial supratentorial tumor that occurs within the first two years of life. Despite the worrisome radiological and histological appearance, the tumors are often curable following gross total resection. Tumors with similar characteristics are exceedingly rare in the noninfantile population. We present a six-year-old boy with seizures, weakness, and unsteady gait. Radiographic imaging confirmed a very large, solid and cystic mass in the right temporal-parietal region. Pathological examination demonstrated a tumor with severe desmoplasia identical to those reported as "desmoplastic infantile ganglioglioma." This case adds to the limited data available for desmoplastic gangliogliomas in the noninfantile population. It is not clear, yet likely, that the noninfantile form of this neoplasm is biologically similar to the infantile form. It is also unclear whether the desmoplastic noninfantile ganglioglioma has characteristics similar to classical ganglioglioma. This rare case highlights the remarkable versatility of glioneuronal tumors in children.