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Follicular dendritic cell sarcoma (FDCS) is an uncommon tumor arising from follicular dendritic cells (FDCs) in the lymph nodes or in extranodal sites. We herein report an unusual case of a 30-year-old man who presented with a FDCS of the omentum and massive liver involvement. The tumor proliferation was a diffuse growth composed by large and highly pleomorphic cells. On immunostaining, B and T cell markers; CD30, myeloid, and histiocytic markers; and S100 were negative whereas CD21, CD23, CD35, clusterin, and fascin were positive. Unexpectedly, large spectrum cytokeratin clone AE1/AE3 was focally and variably expressed. Despite the latter atypical finding and the misleading morphology, immunohistochemistry allowed for a conclusive diagnosis of FDCS.

An unusual case of follicular dendritic cell sarcoma of the omentum with pleomorphous morphology and aberrant cytokeratin expression