Tumors of mixed glioneuronal type are well recognized in the central nervou
s system. The most common examples of these lesions include gangliogliomas
and dysembryoplastic neuroepithelial tumors. Recently, unusual examples of
these lesions have been described, including the papillary glioneuronal tum
or. This report describes a histologically similar-appearing lesion arising
in the left parieto-occipital lobe of an 18-year-old man who presented wit
h headaches and difficulties with vision. Imaging studies noted a large cys
tic neoplasm marked by a peripheral rim of enhancement. The patient underwe
nt gross total resection of the tumor, which histologically was marked by a
mixture of glial (glial fibrillary acidic protein-positive) and neural (sy
naptophysin-positive) components. Architecturally, the tumor was notable fo
r a focal pseudopapillary pattern. Papillae were lined by predominantly gli
al cells, with intervening areas occupied by neurally differentiated cells.
Mitotic activity, vascular proliferation, and necrosis were not noted. A M
IB-1 labeling index of 1.1% was seen. p53 immunoreactivity was not observed
. This report adds further evidence supporting the existence of this unusua
l mixed glioneuronal tumor of the central nervous system.