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A 54 year old woman with a history of melanoma and breast cancer presented with a right-sided nasal mass and symptoms of airway obstruction and sinus pressure, which had progressively worsened over the past several weeks.

Inspection of the right nasal cavity revealed inspissated mucus overlying a whitish-gray mass with friable adjacent mucosa. Nasal endoscopy demonstrated a mass extending from the nasal cavity into the right middle meatus and right ethmoid region. This was biopsied. Representative hematoxylin and eosin stained sections are shown below. Immunohistochemistry was strongly positive for desmin, vimentin, and muscle specific actin. The tumor cells also showed focal positive immunostaining for S100, synaptophysin, and chromogranin. Immunohistochemistry was negative for pancytokeratin, melan-A, CD99, TTF-1, and leukocyte common antigen. Break-apart fluorescence in-situ hybridization was positive for a rearrangement involving the forkhead (FKHR) region (13q14) as reflected by a mixed yellow, red, and green signal pattern.