Evidence for developmental precursor lesions in epilepsy-associated glioneuronal tumors.
The etiology and pathogenesis of epilepsy-associated local lesions remain largely unknown. Histopathologically, the most frequent lesions comprise gangliogliomas and glioneuronal malformations, i.e., hamartias or hamartomas, with a preferred location in the temporal lobe of young patients. A characteristic histopathological admixture of glial and neuronal elements, the focal appearance and the benign clinical behaviour suggest a malformative nature. So far, no molecular genetic alterations specifically involved in the pathogenesis of these glioneuronal lesions have been identified. However, immunohistochemical analysis revealed distinct distribution patterns of oncofetal antigens. The embryonic form of the neural cell adhesion molecule is present within glioneuronal hamartias, indicating an early migrational disorder. Recently, we have observed immunoreactivity for the stem cell marker CD34 in the majority of gangliogliomas and glioneuronal hamartomas. Based on these findings, we propose a common origin of gangliogliomas and glioneuronal hamartomas from a bipotent precursor that undergoes abnormal glioneuronal development.
Department of Neuropathology, University of Bonn Medical Center, D-53105 Bonn, Germany. umt908@uni-bonn.de
I
I
Blümcke
M
M
Löbach
H K
HK
Wolf
O D
OD
Wiestler
D018952:Antigens, CD34 / Q000032:analysis; D000951:Antigens, Neoplasm / Q000032:analysis; D015415:Biomarkers / Q000032:analysis; D001932:Brain Neoplasms / Q000737:chemistry / Q000209:etiology / Q000473:pathology*; D004827:Epilepsy / Q000150:complications / Q000473:pathology*; D018303:Ganglioglioma / Q000737:chemistry / Q000209:etiology / Q000473:pathology*; D006222:Hamartoma / Q000737:chemistry / Q000209:etiology / Q000473:pathology; D006651:Histocytochemistry / Q000379:methods; D006801:Humans; D013702:Temporal Lobe / Q000473:pathology*
D018952:Antigens, CD34; D000951:Antigens, Neoplasm; D015415:Biomarkers; C031192:oncofetal antigens