Cerebellar ganglioglioma in a child

Abstract

A cerebellar ganglioglioma was surgically removed from a two-year old boy, who had developed manifestations of increased intracranial pressure and cerebellar symptoms. At surgery, the tumor presented as a firm nodular mass displacing the cerebellar cortex. By light microscopy, its architecture differed distinctly from that of hamartomatous diffuse hypertrophy of the cerebellar cortex (Lhermitte-Duclos' disease). Mature ganglion cells were grouped in clusters and linked by thick bundles of nerve cell processes. Nerve cells and processes were enmeshed in a rich network of fibrillary connective tissue. Electron microscopy disclosed typical neuronal perikarya as well as numerous asymmetric chemical synapses. The bulk of the tumor consisted of tightly grouped, (non-myelinated) nerve cell processes arranged in parallel. One of the most prominent features of the tumor consisted of numerous dilatations of these processes. The largest ones contained microfilaments, while the smaller ones were entirely filled with dense bodies (most probably derived from degenerating mitochondria). Only scattered dense core vesicles were seen, which probably did not represent neurosecretory granules. A second cell type consisted probably of astrocytes. Most neuroepithelial cell processes could not be identified with certainty as being of either neuronal or glial origin. A third cell type consisted of numerous slender cells which were probably mesenchymal. They were surrounded by a network of basement membrane which extended between the surrounding nerve cell processes.