Primary clear cell sarcoma of the head and neck: a case series with review of the literature

Background: Clear cell sarcoma typically arises in deep soft tissues of the foot/ankle. Primary head and neck clear cell sarcoma is rare. We report three molecularly confirmed primary head and neck clear cell sarcoma and review the literature.

Methods: Head and neck clear cell sarcoma with no primary elsewhere were retrieved from archival/consultation files. English language literature review of all reported head and neck clear cell sarcoma was performed.

Results: Three cases were identified. The tumors, all in men, presented on the scalp of a 33-year-old, face of a 20-year-old and tongue of a 44-year-old. Tumors ranged from 0.6 to 1.4 cm. All had typical features of clear cell sarcoma, including nests of tumor cells divided by fibrous septae. One had multinucleated wreath-like giant cells. One had a focal junctional component. Immunohistochemistry was positive for S100 (three out of three), HMB45 (three out of three) and Melan-A (two out of three). All had EWSR1 rearrangements by fluorescence in situ hybridization. Follow up available for one patient revealed no evidence of disease after wide excision and radiation. Seven additional reported cases were identified and tabulated.

Conclusion: Head and neck clear cell sarcoma is rare but should be considered in the differential diagnosis of nested tumors with fibrous septae. Molecular analysis should be considered for definitive diagnosis regardless of location.