肝滤泡树突状细胞肉瘤一例并文献复习

Hepatic follicular dentritic cell sarcoma: a case report and literature review

目的探讨肝滤泡树突状细胞肉瘤(FDCS)的临床特点。方法回顾性分析2004年郑州大学附属肿瘤医院收治的1例肝FDCS患者临床资料。患者已签署知情同意书,符合医学伦理学规定。患者女,49岁。因上腹部疼痛1月余入院。体检示剑突下可触及12 cm×12 cm肿块,质硬,活动度差,压痛。实验室检查基本正常。CT平扫示肝左叶实性占位,直径约20 cm;增强扫描示动脉期肿瘤不规则强化,延迟期强化减退,肿瘤呈低密度灶。临床诊断为原发性肝癌。结果经过积极术前准备,于2004年8月31日在气管插管全身麻醉下行左半肝切除术。术中见肿瘤位于肝左外叶,大小约23 cm×20 cm,质韧,瘤旁子灶形成,腹腔、盆腔内及腹膜均未见明显转移灶。术后病理学检查示炎性假瘤样FDCS,免疫组化法检查示CD21、CD23、CD35,波形蛋白(VIM)阳性。患者分别于2006年至2014年多次于肝脏、腹腔、胸壁等处发现肿瘤复发转移并行切除术。目前患者病情稳定,定期随访,至投稿日期未见复发或转移。结论肝FDCS是一种极为罕见的疾病,确诊依赖于病理学检查结果。手术切除肿瘤是可靠的治疗手段,预后较好。

Objective To investigate the clinical features of hepatic follicular dentritic cell sarcoma（FDCS）. Methods 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. Results 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. Conclusions 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.