We have established a cultured cell line named FU-UR-1 from a large retroperitoneal tumor of a 24-year-old Japanese male patient who simultaneously had a small renal cell carcinoma (RCC). Cytogenetic analysis and fluorescence in situ hybridization of the retroperitoneal tumor, and the cell line established from this tumor demonstrated similar karyotypes including add(13)(p11).ish der(13)t(13;17) (p11;q11)t(X;17)(p11;q25)(wcpX+,wcp17+). FU-UR-1 had been propagated continuously for more than 70 passages, and the doubling time was 32 h. Successful heterotransplantation was performed by inoculation of the cultured FU-UR-1 cells into the subcutis of BALB/c nude mice. Reverse transcriptase-polymerase chain reaction (RT-PCR) and sequence analysis demonstrated reciprocal ASPL-TFE3 and TFE3-ASPL fusion transcripts in the retroperitoneal tumor, cultured FU-UR-1 cells and xenografted tumors. In addition, the pathological findings of these samples and the renal tumor resembled each other. These observations suggest that the FU-UR-1 cell line established from the retroperitoneal tumor is an RCC cell line. This well-examined cell line may become a useful system for studying the genetic and biologic characteristics of rare neoplasms with the reciprocal ASPL-TFE3 fusion transcript.

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