Clear Cell Tumor with Melanocytic Differentiation and MITF-CREM Translocation - CISMeF
Clear Cell Tumor with Melanocytic Differentiation and MITF-CREM TranslocationNCIt concept
Preferred Label : Clear Cell Tumor with Melanocytic Differentiation and MITF-CREM Translocation;
NCIt synonyms : Clear Cell Neoplasm with Melanocytic Differentiation and MITF-CREM Translocation; CCTMMC; Clear Cell Neoplasm with Melanocytic Differentiation and MITF::CREM Translocation; Clear Cell Tumor with Melanocytic Differentiation and MITF::CREM Translocation;
NCIt definition : 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.;