Abstract
Clear cell sarcoma (CCS), previously known as soft tissue melanoma due to similarities with melanoma, is a rare and aggressive neoplasm. This tumor predominantly occurs in the lower limbs and rarely affects the tongue, as well as other head and neck locations. To our knowledge, only five cases have been reported in the English literature. CCS presents many similar morphological and immunohistochemical features to those of melanomas, sarcomatoid cell carcinoma, angiomatoid histiocytoma, and Ewing sarcoma, which makes the diagnosis difficult, especially in cases of uncommon locations. The treatment is based on oncological surgery and adjuvant radiation therapy as these tumors show low sensitivity to chemotherapy. This study aimed to report a case of an 88-year-old male patient who presented a large, rapidly growing nodular lesion on the right border of the mobile tongue diagnosed with CCS of the tongue.