Histopathological and immunohistochemical profile in anaplastic gangliogliomas

ANTECEDENTES: El ganglioglioma anaplásico (GA) es la contraparte de alto grado del ganglioglioma, un raro tumor compuesto por una mezcla de células neuronales/ganglionares y gliales. MATERIALES Y MÉTODOS: Se describe la histopatología y la inmunohistoquímica en 7casos de GA. RESULTADOS: Los casos de GA correspondieron al 2,5% de los tumores del SNC evaluados en nuestra institución. La edad media de presentación fue 25,7 años, con predominio del género masculino. La presentación más frecuente fue como convulsiones generalizadas tónico-clónicas (3/7casos), en correlación con una frecuente localización cortical/subcortical (6/7casos). Histopatológicamente todos los casos mostraron características de alto grado en las células gliales (GFAPpositivas) y ganglio/neuronales (sinaptofisina, PG-9.5, neurofilamentos, NSE y CD56-positivo), además de celularidad moderada, figuras mitóticas frecuentes y Ki-67>5%. Todos los pacientes mostraron baja supervivencia. CONCLUSIÓN: Se describe el perfil histopatológico e inmunohistoquímico en GA, el cual es constante y posee utilidad para el diagnóstico precoz de estos tumores agresivos

BACKGROUND: The anaplastic ganglioglioma (AG) is the high-grade counterpart of ganglioglioma, a rare mixed tumor composed of neuronal/ganglion and glial cells. MATERIALS AND METHODS: We describe the histopathology and immunohistochemistry in 7 cases of AG and correlate them with the clinical and radiological features. RESULTS: Our AG patients correspond to 2.5% of the central nervous system tumor patients evaluated in our institution. The mean age at presentation was 25.7 years, with a malepredominance. The most common clinical presentation was generalized tonic-clonic seizures (3/7 cases), in correlation with frequent cortical/subcortical location (6/7 cases).Histopathologically, all our cases showed high-grade features in glial (glial fibrillary acid protein-positive) and neuron-ganglion cells (synaptophysin, PGP-9.5, neurofilament, NSE and CD56-positive), as well as moderate cellularity, frequent mitotic figures and a Ki-67labeling index >5%. All our patients had poor survival. CONCLUSION: We found that a typical histopathological and immunohistochemical profile is constant and can be useful in early diagnosis of these aggressive neoplasms

Histopathological and immunohistochemical profile in anaplastic gangliogliomas.

BACKGROUND: The anaplastic ganglioglioma (AG) is the high-grade counterpart of ganglioglioma, a rare mixed tumor composed of neuronal/ganglion and glial cells. MATERIALS AND METHODS: We describe the histopathology and immunohistochemistry in 7 cases of AG and correlate them with the clinical and radiological features. RESULTS: Our AG patients correspond to 2.5% of the central nervous system tumor patients evaluated in our institution. The mean age at presentation was 25.7 years, with a male predominance. The most common clinical presentation was generalized tonic-clonic seizures (3/7 cases), in correlation with frequent cortical/subcortical location (6/7 cases). Histopathologically, all our cases showed high-grade features in glial (glial fibrillary acid protein-positive) and neuron-ganglion cells (synaptophysin, PGP-9.5, neurofilament, NSE and CD56-positive), as well as moderate cellularity, frequent mitotic figures and a Ki-67 labeling index >5%. All our patients had poor survival. CONCLUSION: We found that a typical histopathological and immunohistochemical profile is constant and can be useful in early diagnosis of these aggressive neoplasms.