Sb. Wharton et al., Gliosarcoma with areas of primitive neuroepithelial differentiation and extracranial metastasis, CLIN NEUR, 20(5), 2001, pp. 212-218
We report a case of gliosarcoma with areas of primitive neuroepithelial dif
ferentiation arising in the temporal lobe of a 53-year-old man. The sarcoma
tous component of this tumor was perivascular in its distribution and showe
d expression of factor VIII-related antigen, smooth muscle actin and CD34.
The primitive neuroepithelial component possessed a small cell morphology a
nd showed expression of neuronal antigens. Strong expression of p53 was dem
onstrated throughout the tumor with only focal weak expression of epidermal
growth factor receptor. The tumor developed widespread extraneural metasta
ses 5 months after surgical resection of the primary tumor. Histological ex
amination of the liver metastases showed them to consist predominantly of t
he primitive neuroepithelial component. We believe this to be a novel patte
rn of differentiation in a gliosarcoma which in this case was associated wi
th an aggressive metastatic potential.