Gliosarcoma with ependymal and PNET-like differentiation

A rare case of gliosarcoma which arose in the temporal lobe of a 39-yearold man was reported. The gliomatous area of the tumor showed ependymal differentiation, and also contained immature neuroectodermal tissue resembling a primitive neuroectodermal tumor (PNET) in addition to an ordinary glioblastomatous component. Tumor cells in the PNET-like component were immunoreactive for synaptophysin, CD99, neurogenin 3, and α-internexin, but not for glial fibrillary acidic protein (GFAP), Class III-β tubulin, or Neu N. The mesenchymal area exhibited a compact fascicular proliferation of atypical spindle cells invested by fine reticulin fibrils. In addition, these cells were immunoreactive for Slug and Twist - transcription factors which are involved in the "epithelial-mesenchymal transition (EMT)" phenomenon. Gliosarcomas containing an ependymal or PNET-like component are rare, and to our knowledge, the present case is the first to be reported whose glial element exhibited differentiation toward these two components. The diverse differentiation in the glial element suggests that the tumor most likely originated from primitive neuroepithelial progenitor cells rather than from the neometaplasia of a glioblastoma. The immunoreactivity for transcription factors in the mesenchymal element indicated that EMT might be involved in the pathogenesis of this very rare type of gliosarcoma.