Neuronal and Mixed Neuronal-Glial Tumors of the Central Nervous System

Objective: Neuronal and Mixed Neuronal-glial Tumors of the Central Nervous System are frequently encountered in the neurosurgical practice. Differentiation of neuronal tumors from the more common glial tumors is crucial because neuronal tumors have favorable clinical outcomes and are generally curable with total surgical resection alone, whereas gliomas typically require further chemoradiotherapy depending on their histologic grade and have poor prognosis. At histopathologic analysis, neuronal tumors are usually classified as pure neuronal cell tumors (gangliocytoma, Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma], central neurocytoma) and mixed neuronal-glial tumors (ganglioglioma, desmoplastic infantile ganglioglioma, dysembryoplastic neuroepithelial tumor, ganglioneuroma) In this chapter, we review the WHO classification of each type of neuroepithelial cell tumours and the incidence and distribution. The clinical presentation and neuroimaging will also be briefed, then we describe the specific histopathologic characteristics, immunohistochemestry and genetic suscipitability of the various neuronal tumors of the central nervous system and finally demonstrate the outcome of these lesions.