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Neuronal tumors are a rare subset of central nervous system (CNS) neoplasms that originate from cells with neuronal differentiation. Their pathology is distinct from glial tumors and involves unique histological and molecular features. Here's a comprehensive overview: 🧠 Classification of Neuronal Tumors Neuronal tumors are typically categorized under glioneuronal and pure neuronal tumors. Major types include: Tumor Type Key Features Ganglioglioma Mixed tumor with neoplastic ganglion and glial cells; often in temporal lobe Central Neurocytoma Typically intraventricular; composed of uniform neurocytic cells Dysembryoplastic Neuroepithelial Tumor (DNET) Benign, cortical-based; associated with epilepsy Paraganglioma Neuroendocrine tumor; often found in spinal cord or cauda equina Neuroblastoma (CNS type) Rare, aggressive; resembles peripheral neuroblastoma 🔬 Histopathological Features Cell Morphology Neuronal tumors often show cells with round nuclei, prominent nucleoli, and cytoplasmic processes resembling axons or dendrites. Immunohistochemistry Markers Synaptophysin: Marker of neuronal differentiation NeuN: Nuclear marker for mature neurons Chromogranin and NSE: Neuroendocrine markers GFAP: May be positive in mixed glioneuronal tumors Architectural Patterns Central neurocytomas show a "honeycomb" appearance with perivascular pseudorosettes DNETs exhibit columnar arrangements of cells around mucin-rich pools 🧬 Molecular Pathology Genetic Alterations Gangliogliomas may harbor BRAF V600E mutations DNETs often lack significant genetic mutations, supporting their benign nature Central neurocytomas may show chromosome 1p and 19q deletions Epigenetic Profiling Advances in DNA methylation profiling have refined classification and diagnosis. 🧠 Clinical Relevance Symptoms Often present with seizures, especially in cortical tumors like DNET and ganglioglioma. Prognosis Generally favorable for benign types; aggressive variants like CNS neuroblastoma require intensive therapy. Treatment Surgical resection is often curative for benign tumors. Adjuvant therapy may be needed for high-grade or recurrent cases. #Neurons