Clear Cell Sarcoma of the Parotid Gland: Case Report and Review of the Literature

Abstract

Clear cell sarcoma (CCS) of the parotid gland is a rare and aggressive cancer, predominantly affecting the deep soft tissues of the lower extremities, and is more frequently observed in the elderly. Its occurrence in the head and neck area, particularly in the parotid gland, is exceedingly rare. This study presents a 75-year-old male who exhibited a substantial, swiftly-enlarging mass on the left side of the mandible, which was diagnosed as CCS of the parotid gland. Morphologically and immunohistochemically, CCS shares characteristics with melanomas, and Ewing sarcoma, complicating differential diagnosis. The primary treatment for localized CCS typically involves surgical excision with clear margins. However, due to its resistance to conventional chemotherapy, there is ongoing research regarding the efficacy of adjuvant therapies such as chemoradiation, lymphadenectomy as well as immunotherapies such as immune checkpoint inhibitors. Further research is imperative to enhance our understanding of CCS and to develop more effective treatment strategies.

Keywords: EWSR1 rearrangements; clear cell sarcoma; head and neck; parotid gland.