In 2024, Argani et al. also proposed renaming these tumors as “TFE3-rearranged PEComa-like neoplasms”, highlighting the younger age of the patients, the association with prior exposure to chemotherapy, and the almost constant lack of muscle marker expression compared to TSC-related PEComas [78]. The gene discussed is TSC1; the disease is neoplasm with perivascular epithelioid cell differentiation.