Clear Cell Tumors of Soft Tissue

Clear cell lesions of soft tissue include varying morphologic patterns and a range of clinical behaviors and prognoses. Benign lesions include perivascular epithelioid cell tumors, clear cell fibrous papule, and distinctive dermal clear cell mesenchymal tumor; malignant tumors include clear cell sarcoma, liposarcoma, and rare malignant perivascular epithelioid cell tumors. Clear cell variants of other benign and malignant soft tissue tumors include fibrous histiocytoma, atypical fibroxanthoma, myoepithelioma, leiomyoma and leiomyosarcoma, and rhabdomyosarcoma. Metastatic clear cell tumors, including renal cell carcinoma and adrenal cortical carcinoma, should be considered in the differential diagnosis and excluded through clinical history, imaging studies, and immunohistochemical stains.