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Background:   Ganglioglioma (GG) and dysembryoplastic neuroepithelial tumor (DNET) belong to the group of low-grade epilepsy-associated tumors (LEAT) and are the most prevalent tumor types found in patients undergoing epilepsy surgery. Histopathological differentiation between GG and DNET can be difficult on biopsies due to limited tumor tissue.    Case Description:   Here, we present a rare case where a low-grade tumor was initially classified as DNET, based on biopsy findings and unfortunately dedifferentiated within 10 years into a glioblastoma multiforme. After gross total resection, the initial tumor was reclassified as GG.    Conclusion:   This case illustrates the diagnostic challenges of LEAT, especially on biopsy material. Therefore, we advocate to counsel for complete resection and histopathological diagnosis utilizing tumor markers to confirm the nature of the tumor and to advice type of follow-up and eventual concurrent treatment.

From a dysembryoplastic neuroepithelial tumor to a glioblastoma multiforme: Pitfalls of initial diagnosis on biopsy material, a case report