Abstract
Follicular dendritic cell sarcoma (FDCS) is a rare entity, often presenting a diagnostic challenge for both the pathologist and the clinician. It accounts for only 0.4% of soft-tissue sarcomas, and its underlying causes are largely unknown. Most of these tumours occur in lymph nodes, and extranodal involvement is uncommon. In the gastrointestinal tract, FDCS is extremely rare. Here, we report a case of primary FDCS originating in the stomach. Upon review of the literature, we identified only 2 additional cases of FDCS presenting as a primary stomach tumour. Given the rarity of this tumour in gastrointestinal sites and the lack of consensus on treatment, evaluation of this entity must continue.