探讨肝滤泡树突状细胞肉瘤（FDCS）的临床病理学特征及其诊治。

回顾性分析2015年12月中山大学孙逸仙纪念医院收治的1例肝FDCS患者临床资料，并复习相关文献。患者已签署知情同意书，符合医学伦理学规定。患者男，年龄34岁，因体检发现肝左叶占位性病变10 d入院。无肝炎、肝硬化病史。体检：全身皮肤、巩膜无明显黄染，浅表淋巴结未触及肿大，肝脾肋下未触及，肝区无明显叩击痛。实验室检查：ALT 162 U/L、AST 74 U/L、GGT 148 U/L，AFP、CEA、CA19-9、癌抗原（CA）125、CA72-4正常。超声、CT、MRI检查示：肝左外叶占位性病变。入院诊断为原发性肝癌。

患者入院3 d行腹腔镜左半肝切除术，术中见肿瘤位于肝左外叶，切面灰黄色。术后病理学检查示梭形细胞和卵圆细胞弥漫性增生，淋巴细胞、浆细胞、中性粒细胞及嗜酸性粒细胞浸润。免疫组化检测示：Vimentin（+）、CD21（+）、CD35（+）、CD23（+）、EBER（+），病理学诊断高分化肝FDCS。患者术后定期随访，截止投稿日期未见肿瘤复发。

肝FDCS无特异性临床表现，确诊主要依靠病理学检查，免疫组化在鉴别诊断中有重要作用。治疗以手术切除为主。

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.