Xp11.2 translocation/TFE3 gene fusion-related renal cancer often affects young people, and lymph node metastasis is also common, with a good short-term prognosis. This article reports a 19-year-old young female with a huge metastatic Xp11.2 translocation/TFE3 gene fusion-related renal carcinoma, which metastasize to lung and lymph node. The patient underwent axitinib neoadjuvant targeted therapy before the operation. During the operation, lymph node metastasis was found and the para-aortic lymph node dissection was performed.After the operation, the patient continued to be treated with axitinib, and the lung metastases improved. There was no local recurrence and metastasis after 1 year follow-up.
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