TFE renal cell carcinoma (TFE RCC) (otherwise known as translocation renal cell carcinoma- tRCC) is a specific subtype of renal cell carcinoma that is caused by a translocation mutation between two different genes, causing an overexpression of genes and cancer cells to grow. TFE RCC is still a relatively new disease, being formally recognized by the WHO in 2004. As such, much about its biological behavior is unknown.
Although it is a rare type of cancer, TFE RCC accounts for up to 5% of adult RCCs and approximately 1/2 of all pediatric RCCs with it predominately found in children and young adults. Currently, there are no formal treatment recommendations available for TFE RCC and no dedicated therapeutic trials have been conducted. Limited retrospective studies have been performed previously, but more data gathered over a long term period of time and from a large number of patients is needed to gain better insight into TFE RCC. The TRRI registry is aimed to provide this long term follow up needed to better understand prognosis and outcomes of disease, and the consortium to conduct hypothesis driven research to develop better treatments options by developing tumor models and performing drug tests and screenings.