Establishment and Characterization of a TFE3-rearranged Renal Cell Carcinoma Cell Line (FU-UR-2) With the PRCC-TFE3 Fusion Transcript

Background/Aim: Xp11.2-RCC was classified into molecularly defined renal carcinomas and named TFE3rearranged renal cell carcinoma (TFE3-rRCC) in the 2022 World Health Organization classification of renal tumors. Materials and Methods: In this study, we established and characterized a TFE3-rRCC cell line from a right-sided renal tumor of a 35-year-old female patient and named it FU-UR-2. FU-UR-2 had been initially diagnosed as a papillary RCC because the patient was 35 years old, a routine immunohistochemical staining for TFE3 was negative, and its morphology was papillary. The G-band analysis revealed an X-chromosome aberration, thus we performed immunohistochemical re-staining for TFE3 and examined the aberration in the TFE3 gene by reverse-transcriptase polymerase chain reaction and fluorescence in situ hybridization. Results: FUUR-2 was confirmed as a TFE3-rRCC with a PRCC-TFE3 fusion transcript. Conclusion: Cultured FU-UR-2 cells continuously propagated over 90 passages and may provide a new permanent culture model to study pathogenetic mechanisms, investigate biological behavior, and develop new treatments such as molecular-targeting antitumor agents or immunological drugs for TFE3-rRCCs.