ABSTRACT
Within the neuronal and mixed neuronal-glial tumors group, several are known to affect neurons of the central nervous system (CNS). These tumors consist of gangliocytoma, Lhermitte–Duclos disease (LDD), ganglioglioma, and desmoplastic infantile ganglioglioma. Patients with recurrent gangliocytoma are found to harbor mutation in the TP53 gene but not in the epidermal growth factor receptor (EGFR) gene. Depending on the tumor's location, clinical symptoms of gangliocytoma may range from seizures, increased brain pressure, endocrine disorders, focal symptoms, anxious/depressed mood, insomnia, fatigue, and pain to no symptom. Differential diagnosis of ganglioglioma includes astrocytoma, ependymoma, hemangioblastoma, and paraganglioma. Complete resection of supratentorial gangliocytomas is achievable in less than 75" of cases, and clinically relevant recurrence/regrowth of the tumor is rare even after partial resection. Treatment for symptomatic LDD patients includes surgical debulking of the tumor, if complete resection is not possible.
