In NCCN guidelines and clinical practice, patients with HLRCC will be treated with conventional clear cell carcinoma medications, such as vascular endothelial growth factor receptor (VEGFR) tyrosine kinase inhibitors (TKIs), mammalian target of rapamycin (mTOR) inhibitors, and immune checkpoint inhibitors (14, 15). Here, MTOR is linked to hereditary leiomyomatosis and renal cell cancer.