To investigate the clinicopathological characteristics, diagnosis and treatment of hepatic follicular dendritic cell sarcoma (FDCS).

Clinical data of one case with hepatic FDCS who was admitted to Sun Yat-sen Memorial Hospital of Sun Yat-sen University were retrospectively analyzed and relevant literature was reviewed. The informed consents of the patient were obtained and the local ethical committee approval was received. The male patient, aged 34 years old, was admitted to hospital due to an occupying lesion in the left lobe of liver found by physical examination for 10 d. The patient had no history of hepatitis or liver cirrhosis. No evident yellow skin or sclera was found by physical examination. No palpable superficial lymphadenopathy was found. The liver and spleen were not palpated under the ribs. No apparent percussion pain was found in the liver area. Laboratory test revealed the levels of alanine aminotransferase (ALT) was 162 U/L, aspartate aminotransferase (AST) was 74 U/L and gamma-glutamyl transferase (GGT) was 148 U/L. And the alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), cancer antigen (CA) 125, and CA72-4 were within the normal range. An occupying lesion in the left lateral lobe of liver were found by Ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI). The admission diagnosis was primary liver cancer.

Larparoscopic left hemihepatectomy was performed on the patient at 3 d after admission. A tumor with grayish yellow section was found in the left lateral lobe of liver during the operation. Postoperative pathological examination revealed diffuse proliferation of spindle and oval cells, and infiltration of lymphocyte, plasma cell, neutrophil and eosinophil. Immunohistochemical test revealed Vimentin(+), cluster of differentiation (CD)21(+), CD35(+), CD23(+) and EBER(+). The pathological diagnosis was highly-differentiated hepatic FDCS. The patient received a regular follow-up after operation and no tumor recurrence was observed until the submission date.

Hepatic FDCS has no specific clinical manifestation. The diagnosis depends on pathological examination. Immunohistochemical test plays an important role in the differential diagnosis. And the mainly treatment was surgical resection.