Nerve cell tumours of the cerebrum: variable clinical and pathological manifestations.

Nerve cell tumours of the cerebrum tend to display a high degree of morphological variability from case to case, and this leads to poor understanding of these tumours. We retrospectively reviewed the clinical and patho-anatomic features of 16 primary nerve cell tumours of the cerebrum (M:9; F:7; average age at onset: 10.2 years). Intraventricular tumours were not included. In 13 patients epileptic seizures were the only symptoms, while three had headache or hemiparesis. Seven tumours were located in the frontal lobe, four in the parietal lobe, two in the temporal lobe and one each in the fronto-parietal lobes, occipital lobe and the midbrain. Tumours were histologically classified into three groups. In the first group, six tumours had the morphological features of classic gangliocytoma or ganglioglioma. In the second group six cerebral and midbrain tumours were composed of small cells, which showed apparent neuronal differentiation including positive immunoreactivity for synaptophysin and the presence of synaptic structures. These tumours usually involved both the cortex and white matter. In the third group, three tumours were composed of small nerve cells and ganglioid cells. All tumours were relatively well circumscribed, and thus eight tumours were totally removed, five subtotally and three partially. Following surgery, three patients, except one, are alive with stable imaging findings for 4 months - 19.3 years (average 11.6 years) after treatment. While small nerve cell tumours are found throughout the cerebrum and its identification broadens the spectrum of neuronal and mixed neuro-glial tumours, most of these tumours are biologically indolent. Copyright 2001 Harcourt Publishers Ltd.