Central neurocytoma is a rare neuronal tumor affecting young adults and usu
ally located in the lateral ventricles. Postoperative prognosis is generall
y good. Histologically, central neurocytoma is composed of isomorphous smal
l round or ovoid cells alternating with irregularly shaped patches of fibri
llary matrix similar to the neuropile, In a series of 10 cases, two central
neurocytomas were histologically "atypical" at first examination. One was
intra-ventricular. and the second had an intra-parenchymatous juxta-ventric
ular location. Both were highly cellular with mitotic activity, and tumor n
ecrosis was seen in one. Neuronal differentiation was assessed by synaptoph
ysin immunoreactivity in all 10 cases and by ultrastructural examination in
four, including the two "atypical " forms. Neuronal differentiation was le
ss marked in these "atypical" forms, one also presenting focal GFAP immunor
eactivity, The proliferative potential was determined by MIB-1 labeling ind
ex and compared with clinical outcome, The eight classical central neurocyt
omas had a MIB-1 labeling index < 2.3 %, whereas the two "atypical" forms h
ad a MIB-1 labeling index > 5.2 % and both recurred.
We think that there is a spectrum of small-cell neuronal tumors. The two ex
tremes could be the central neurocytoma and the primary cerebral neuroblast
oma, while the intermediate forms might be qualified as "atypical neurocyto
ma". In our series, the histological and immunohistochemical criteria of bi
ological aggressiveness appeared to be high mitotic activity, tumor necrosi
s, loss of neuronal differentiation and high MIB-1 labelling index.