Malignant supratentorial ganglioglioma (ganglion cell-giant cell glioblastoma) - A case report and review of the literature

Background.-From both epidemiologic and pathologic viewpoints, gangliogliom as exhibiting components of giant cell glioblastomas are extraordinary neop lasms. We report herein the case of a 6-year-old girl who presented initial ly with a World Health Organization grade IV anaplastic ganglioglioma (a mi xed ganglion cell tumor-giant cell glioblastoma). Despite aggressive manage ment, the patient died of disease in a relatively short period. Methods.-Formalin-fixed, paraffin-embedded tissue blocks were sectioned at 5 mu m for histochemical and immunohistochemical analyses. Hematoxylin-eosi n-stained sections and immunohistochemically stained sections from the prim ary and secondary resections were reviewed. Reactivity for glial fibrillary acidic protein, neurofilament protein, synaptophysin, and Ki67 nuclear ant igen was evaluated. Results.-Histologically, 2 distinct cell populations we re noted on both the primary and secondary resections. The primary resectio n revealed a neoplasm having a predominant glial component consistent with a glioblastoma. Interspersed were dysmorphic ganglion cells supporting a di agnosis of ganglioglioma. The second resection (following therapy) demonstr ated a much more prominent dysmorphic ganglion cell component and a subdued glial component. Conclusion.-Although immunohistochemical analysis clearly distinguished the 2 tumor cell populations, the identification of Nissl substance in neurons proved to be equally helpful. Although other cases of grade III gangliogli omas and rare cases of grade IV gangliogliomas have been reported, the pres ent case is exceptional in that, to our knowledge, it is the only report of a patient who presented initially with a composite grade IV ganglioglioma and who was clinically followed up to the time of death. This case allows d irect comparison between the histologic findings in a giant cell glioblasto ma and a ganglioglioma and documents the aggressive biologic behavior of th is complex neoplasm.