Hyalinizing Clear Cell Carcinoma of the Maxilla

Hyalinizing clear cell carcinoma (HCCC) is a rare low-grade salivary gland tumor with distinctive clear cell morphology, accounting for < 1% of all salivary gland tumors. In the majority of cases, the tumor originates typically from the minor salivary glands in the oral cavity. A total of 155 cases of HCCCs from head and neck region have been reported in the literature until 2017, of which only 16 are of maxillary origin. Due to its low incidence, there is no clear consensus on prognostic factors and optimal treatment strategies yet. In this paper, a new case of HCCC of the maxilla is presented and its clinical and histopathological features are discussed.