Ganglioglioma

Abstract

Ganglioglioma is a rare, mixed neuronal-glial neoplasm of the central nervous system that occurs in young patients and has a benign clinical course. To define the immunophenotypic and morphologic features of ganglioglioma precisely, 27 specimens were studied by routine histochemistry, 21 specimens by immunochemistry, and 14 specimens were examined at the ultrastructural level. The age of the 27 patients, 14 males and 13 females, ranged from 3 to 52 years (mean, 22 years). The most commonly affected site was the temporal lobe (13 patients). Three patients experienced a local recurrence. Microscopically, the tumors were comprised of well differentiated, somewhat abnormal neurons as well as glial cells, the latter including astrocytes of fibrillary (59%) and pilocytic (41%) type. Scant mitotic activity was observed in 2 tumors (7%). Glial cells of all tumors were immunoreactive for glial fibrillary acidic protein, S-100 protein, and vimentin. Ki-67 labeling indices (LI) ranged from 0.6 to 10.5% (mean, 2.7%) and p53 LI from 1.1 to 42.4% (mean, 15.6%). Ki-67 and p53 LI in recurrent tumors were significantly higher than those of nonrecurrent ones (P = 0.036 and 0.026, respectively). No examples of anaplastic transformation were encountered. Immunohistochemically, many neuronal cells were positive for synaptophysin (100%), Class 3 beta-tubulin (100%), neurofilament protein (90%), and chromogranin A (86%), in addition to S-100 protein (71%) and, occasionally, vimentin (24%). Ultrastructural characteristics of neuronal cells included the presence of numerous, 100-230-nanometer dense core granules within both perikarya and cell processes, well developed rough endoplasmic reticulum, microtubules within cell processes, and synapses associated with clear vesicles. Astrocytic cells usually contained abundant intermediate filaments; their cell membranes, when abutting the stroma, were covered by basal lamina. Gangliogliomas are comprised of well differentiated neuronal cells and glial cells that are very often of pilocytic type. No cells with features intermediate between neurons and glia were observed. Neuronal cells are characterized by prominent neurosecretory features distinct from those of normal neurons in the central nervous system. Higher Ki-67 and p53 LI may indicate more aggressive behavior.