Inflammatory pseudotumor-like follicular dendritic cell sarcoma of the liver

Inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) is a rare, low-grade, malignant tumor that mainly involves the spleen and liver, is associated with Epstein-Barr virus and is predominant in middle-aged females. Its histopathology is similar to that seen in inflammatory pseudotumor, but their immunophenotype is different and IPT-like FDCS does not respond to anti-inflammatory drugs. Surgery is the treatment of choice, and chemotherapy or radiation therapy is applied for advanced tumors. A high index of suspicion is needed in presence of large size lesions in the liver or spleen that exhibit a relatively indolent clinical course.