Report describes primary clear cell sarcoma of head, neck
Patrick C. Feasel, M.D., from the Cleveland Clinic, and colleagues reported three cases of molecularly confirmed primary head and neck clear cell sarcoma, with no primary elsewhere.
The researchers found that the tumors presented on the scalp of a 33-year-old man, the face of a 20-year-old man, and the tongue of a 44-year-old man. The tumors ranged in size from 0.6 to 1.4 cm. Typical features of clear cell sarcoma, including nests of tumor cells divided by fibrous septae, were seen in all tumors, while one had multinucleated wreath-like giant cells and one had a focal junctional component. All three tumors had immunohistochemistry for S100 and HMB45, and two had immunohistochemistry for Melan-A. By fluorescence in situ hybridization, all had EWSR1 rearrangements. In one patient, follow-up showed no evidence of disease after wide excision and radiation. Seven additional cases were identified on review of the literature.
"Head and neck clear cell sarcoma is rare but should be considered in the differential diagnosis of nested tumors with fibrous septae," the authors write. "Molecular analysis should be considered for definitive diagnosis regardless of location."
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