label: Clear Cell Tumor with Melanocytic Differentiation and MITF-CREM Translocation
label_lc: clear cell tumor with melanocytic differentiation and mitf-crem translocation
description: A MITF pathway-activated melanocytic tumor characterized by a cytogenetic translocation between MITF and CREM genes that results in MITF-CREM gene fusion. It presents as a single non-pigmented cutaneous nodule in the distal extremities. It is composed of large cells with clear cytoplasm that exhibit melanocytic differentiation and proliferate in the dermis without an intraepidermal component. Extension in the subcutaneous tissue is frequently seen. The neoplastic cells exhibit atypical features, including nuclear enlargement and hyperchromasia, irregular nuclear borders, and enlarged nucleolus. Mitotic activity is prominent. The biologic potential of this tumor is unknown.