Inflammatory pseudotumor-like follicular dendritic cell sarcoma: a rare presentation of a hepatic mass

Follicular dendritic cell (FDC) sarcoma is a rare, low-grade malignant tumor originating from follicular dendritic cells in germinal centers that accounts for 0.4% of all soft tissue sarcomas. FDC sarcoma is classified into two types, the classic FDC sarcoma and inflammatory pseudotumor (IPT)-like follicular dendritic cell (FDC) sarcoma, the latter of which is rarer. IPT-like FDC sarcoma mainly involves the spleen and liver with non-specific clinical and imaging manifestations. It is often misdiagnosed as an inflammatory disease such as a liver abscess or a malignant tumor such as hepatocellular carcinoma, with a pathological morphology similar to inflammatory pseudotumors. IPT like FDC sarcoma mainly consists of a large number of inflammatory and round, oval and spindle cells with less pleomorphism. These tumor cells are arranged in a whorled, storiform, or sheet pattern. The immunophenotype of IPT-like FDC sarcoma is the same as that of FDC sarcoma and is positive for CD21, CD23, and CD35, and positive for EBER in situ hybridization (ISH). This disease is easily misdiagnosed because it is so rare that clinicians and pathologists may not consider it in diagnosis. Here, a case of IPT-like FDC sarcoma in the liver was reported, and the related literature was reviewed to summarize the clinicopathological features, treatment, and prognosis of this rare new type of FDC sarcoma, providing new knowledge of this rare neoplasm.