Clear cell odontogenic carcinoma with skeletal metastasis: A rare case report

Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic malignancy with a female preponderance occurring in the adult age group. CCOC was classified as a malignant neoplasm of odontogenic origin by the World Health Organization in 2005 because of its aggressive nature, local recurrence tendency, and the potential for regional and distant metastasis. Histologically, CCOC is characterized by sheets and islands of vacuolated and clear cells. Metastasis at presentation is rare; it usually involves cervical lymph nodes and lungs, less frequently to bone. As clear cells are present in few odontogenic tumors; salivary gland neoplasms; and metastatic tumors to the jaws, especially metastatic renal cell carcinoma, the presence of clear cells in a lesion of the head and neck region poses a diagnostic challenge. So knowledge about the clinical course, histopathological pattern, and immunoprofile of CCOC helps in differentiating the other clear cell tumors.