Management of exceptional tumor: MITF::CREM-rearranged tumor on the scalp

A 57-year old woman presented with a 18-month history of a vertex lesion. The first excisional biopsy showed an histopathology dermal proliferation of epithelioid splinded cells, arranged in nests or trabeculae, suggesting a tumoral pathology such as sarcoma or melanoma, without a definitive diagnosis. Further surgical treatment was realised. The definitive diagnosis is a clear cell tumor with melanocytic differentiation and MITF::CREM translocation. It is a recently described rare tumor, with less than 5 cases reported. This article addresses the management of a rare tumor in a plastic surgery ward.