TFE3 acts as an oncogene which is commonly rearranged in many tumour types, such as translocation of Xp11.2 leading to renal cell carcinoma, alveolar soft tissue sarcoma (ASPS), a subset of perivascular epithelioid cell tumours (PEComas), and partial epithelioid hemangiosarcoma [15, 16]. Here, TFE3 is linked to neoplasm with perivascular epithelioid cell differentiation.