Lipomatous supratentorial primitive neuroectodermal tumor with glioblastomatous differentiation

Abstract

Cases of cerebral neuroblastoma or supratentorial primitive neuroectodermal tumor with malignant glioblastomatous components are relatively uncommon. Less frequent is the combination of these two elements with a mesenchymal component. We report a case of lipomatous supratentorial primitive neuroectodermal tumor with glioblastomatous differentiation occurring in a 48‐year‐old woman. She presented with headaches and confusion. A right parietal lobe mass was excised and subsequently recurred, requiring additional surgery ten months later. The patient died 13 months after initial surgery. Histology showed a proliferation of small, rounded, synaptophysin‐positive‐staining neural cells consistent with neuroblastoma. These cells were arranged against a benign lipomatous background. The second resection consisted primarily of glioblastomatous‐like tissue with an intermixed lipomatous component. The glioblastoma component was marked by prominent cellularity, moderate nuclear pleomorphism, readily identifiable mitotic activity, vascular proliferative changes, and necrosis. The glioblastomatous component of the tumor demonstrated GFAP immunoreactivity. A Ki‐67 labeling index of 18.9% was noted in the initial resection. The case serves as a focus for discussion on the relationship of the various components (embryonal, mesenchymal and glial) of this tumor.