Low-grade clear cell carcinoma with myoepithelial features in the submandibular gland.

Clear cell carcinoma is rarely found in the salivary gland. It is classified as a low-grade carcinoma. This case demonstrates a low-grade clear cell carcinoma with myoepithelial features in the submandibular gland which differs from hyalinizing clear cell carcinoma and epithelial-myoepithelial carcinoma. A 32-year-old man presented with a 7 month history of left submandibular swelling. Left submandibular gland excision and left-sided supra-omohyoid neck dissection were performed. Microscopically, the tumor was circumscribed and composed predominantly of cords and nests of clear ovoid cells, set in a densely hyalinizing stroma. These cells are diffusely immunoreactive for cytokeratin AE1/AE3 and focally reactive for vimentin and smooth muscle actin (SMA). Based on these findings, the tumor was diagnosed as "a low-grade clear cell carcinoma with myoepithelial features". The post-operative course was uneventful and the patient is free from disease 21 month after surgery.