Report Describes Primary Clear Cell Sarcoma of Head, Neck

Three cases described in males on scalp, face, tongue; all had nested tumors with fibrous septae


MONDAY, June 13, 2016 (HealthDay News) -- Three cases of primary clear cell sarcoma of the head and neck have been described in three male patients, according to a review published online June 6 in the Journal of Cutaneous Pathology.

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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