Follicular dendritic cell sarcoma of the liver: Unusual presentation of a rare tumor and literature review

BACKGROUND: Hepatic follicular dendritic cell (FDC) sarcoma is an extremely rare neoplasm. Most commonly, FDC sarcoma presents as a solitary mass in lymph nodes, however, several extra-nodal locations have been identifed. METHODS: We report a case of a 53-year-old female who presented with symptoms of abdominal pain, fever, anemia, and jaundice. After an extensive review of the literature, we have found only 12 cases of hepatic FDC sarcoma. RESULTS: The tumor was 11.5 cm in diameter and composed of spindle and epithelioid cells with ovoid nuclei and associated with mixed infammatory infltrate. Immunohistochemical stains were positive for CD35 and CD21. The patient underwent a left hepatic lobectomy. CONCLUSIONS: Liver follicular dendritic cell sarcoma is a very rare tumor. Most cases present with abdominal pain and weight loss, and most of them can be managed by hepatic resection with excellent short-term outcomes.