The papillary glioneuronal tumor is a WHO grade 1, rare neuronal-glial tumor and comprises 0.02% of all CNS tumors. Histologically, it is a mixture of glial and neuronal components showing a pseudopapillary pattern with hyalinized vessels. PGNT is considered a low-grade neoplasm, and surgical excision has been curative in most cases. In this paper, we report a new case of papillary glioneuronal tumor in a 44-year-old male having a divergent presentation, to analyze it due to the rarity of its occurrence as per the latest classification.
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