Primary hepatic Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma: a rare case with genomic profiling and therapeutic implications

Abstract Hepatic Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+IFDCS) is an extremely rare low-grade malignant tumor. Due to its rarity and lack of specific symptoms, laboratory markers, or characteristic imaging features, this disease is frequently misdiagnosed. A 50-year-old woman was diagnosed with left hepatic mass and underwent laparoscopic left hemihepatectomy. The postoperative histopathology confirmed EBV+IFDCS. To our knowledge, we conducted the first-ever genetic testing for hepatic EBV+IFDCS. The analysis indicates that the patient has a low tumor mutational burden and low microsatellite instability, with no identifiable targetable gene mutations, but may have potential sensitivity to certain chemotherapy agents. Postoperatively, the patient was empirically treated with six cycles of S-1 chemotherapy (60 mg twice daily, 2 weeks on/1 week off), and the recovery process was uneventful. We hope that our findings may contribute to the understanding and clinical management of this disease.