Follicular Dendritic Cell Sarcoma : Epidemiology

Comments:
Epidemiology: Follicular dendritic cell sarcoma (FDCS) is an extremely rare tumor. It occurs mainly in young or middle-aged adults. The median age at presentation is 50 years with no gender predilection. Rare cases have been reported in children.

Associations: About 10% to 20% of FDCS cases are associated with Castleman's disease (usually of hyaline vascular type; rarely the plasma cell variant). A small subset of patients with both FDCS and Castleman's disease also have paraneoplastic pemphigus. Schizophrenia is another disease associated with FDCS. There is no evidence of involvement of human herpesvirus 8 or EBV (except in some cases with inflammatory pseudotumor-like features occurring in liver and spleen).

This image shows FDCS arising in colon. There is proliferation of a uniform population of spindle cells in fascicular, storiform, and whorled patterns. The spindle cells are admixed with an inflammatory infiltrate of small lymphocytes and plasma cells.