To investigate the clinical features of hepatic follicular dentritic cell sarcoma (FDCS).

Clinical data of a patient with hepatic FDCS treated in Cancer Hospital Affiliated to Zhengzhou University in 2004 were retrospectively analyzed. The informed consent of the patient was obtained and the local ethical committee approval had been received. The patient, female, 49-year-old, was admitted to the hospital for the complaint of abdominal pain for 1 month . A 12 cm×12 cm in diameter, hard, poor activity tumor was found below xiphoid by physical examination. Abdominal tenderness was positive and laboratory examinations were essentially normal. CT scan showed a 20 cm in diameter solid lesion in the left lobe of liver. The tumor was observed irregularly enhanced in the arterial phase by enhanced CT scan. The enhancement faded away in the delayed phase, and the tumor revealed a low-density lesion. The initial diagnosis was primary liver cancer.

After an active preoperative preparation, the patient underwent left lobectomy under endotracheal general anesthesia on August 31, 2004. A tough tumor measuring 23 cm×20 cm in diameter was found in the left lobe of liver during the surgery. Nodule foci were found beside the tumor and no obvious metastasis was found within the abdomen, pelvic cavity and peritonium. Postoperative pathological examination indicated inflammatory pseudotumor FDCS and Immunohistochemistry indicated positive CD21, CD23, CD35 and vimentin (VIM). Tumor recurrence and matastasis were observed in the liver, abdomen cavity and chest wall etc. repeatedly and was resected during 2006 to 2014. The patient is currently in stable condition and no recurrence or metastasis was observed during regular follow-up till submission date.

Hepatic FDCS is a very rare disease. The diagnosis relies on the results of pathological examination. Surgical resection is a reliable treatment and the prognosis is favorable.