BACKGROUND: Lipoblastoma-like tumors (LLTs) are uncommon lipogenic neoplasms that predominantly occur in the vulva of female individuals. This study presents a novel case of LLT located on the tongue with an IDH1 mutation. Both the site of occurrence and molecular alterations are reported for the first time. CASE PRESENTATION: A 65-year-old female patient presented to the hospital 1 month ago with an incidental finding of a multinodular mass at the right lingual margin. We performed an incision along the outer edge of the mass and completely excised it 0.5 cm beyond its periphery. The final pathological diagnosis after routine examination confirmed a lipoblastoma-like tumor. Furthermore, Next-generation sequencing revealed the presence of mutations in IDH1. CONCLUSIONS: This study presents the first documented case of LLT, thereby expanding its anatomical site of tongue and confirming the presence of a significant IDH1 mutation, thus enhancing our understanding of the molecular alterations in LLT.
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