How do you treat patients with newly diagnosed metastatic MiT Family Translocation Renal Cell Carcinoma?

The AREN1721 clinical trial is accepting patients of all ages with unresectable or metastatic TFE/translocation renal cell carcinoma (RCC). The tumors must have either TFE3 expression by immunohistochemical staining or evidence of a TFE3 or TFEB translocation. The three arm trial compares nivolumab, axitinib, or the combination, with treatment up to two years.

I recently saw a patient with an MiT family translocation RCC who also had t(6;11), which is very rare, who enrolled on the trial. If a p...

Data regarding systemic therapy for patients with metastatic translocation (TFE3 translocation) RCC is limited to observational studies. For first line therapy, I will suggest clinical trial or VEGF-directed therapy such as sunitinib (Malouf et al., PMID 20154303, Choueiri et al., PMID 20665500) or cabozantinib (https://ascopubs.org/doi/abs/10.1200/JCO.2021.39.6_suppl.274).