Follicular dendritic cell sarcoma (FDCS) of the liver is an extremely rare disease, accounting for a mere 0.4% of all soft tissue sarcomas. FDCS most commonly involves lymph nodes but also affects extranodal sites such as the gastrointestinal system, oral cavity, liver, spleen and pancreas, albeit less commonly. It is widely considered a low-to-intermediate grade malignancy. We report a case of FDCS with metachronous involvement of the liver, small intestines and spleen, its imaging, histological findings and its management.
Keywords: Follicular dendritic cell sarcoma (FDCS); extra-nodal; liver.