Case Report: Extrarenal TFE3 fusion-related renal cell carcinoma

The diagnosis of <i>TFE3</i>-RCC requires a comprehensive evaluation of histological features, immunohistochemical markers, and molecular testing. <i>PRCC</i>-<i>TFE3</i> RCC is highly aggressive with a high recurrence rate and poor prognosis in adults. Surgical resection is the primary treatment for localized lesions. However, close follow-up is necessary owing to a high risk of recurrence and metastasis. Targeted therapies and immunotherapies are potential treatment options for patients with advanced or metastatic disease.