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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 dendritic cell sarcoma (FDCS). Methods The clinical data of one patient with liver FDCS admitted to Cancer Hospital of Zhengzhou University in 2004 were retrospectively analyzed. Patients have signed informed consent, in line with medical ethics rules. Female patient, 49 years old. Due to upper abdominal pain more than 1 month admission. Physical examination shows that the xiphoid can reach 12 cm × 12 cm mass, hard, poor mobility, tenderness. Laboratory tests basically normal. CT plain showed the left hepatic lobe solid occupies a diameter of about 20 cm; enhanced scan showed arterial irregular enhancement of the tumor, delayed enhancement decreased, the tumor was low density lesions. Clinical diagnosis of primary liver cancer. Results After positive preoperative preparation, on August 31, 2004 under general anesthesia for endotracheal intubation underwent a left hepatectomy. Surgery, see the tumor is located in the left hepatic lobule, the size of about 23 cm × 20 cm, quality and tough, adjacent tumor formation, peritoneal, pelvic and peritoneal no significant metastases. Postoperative pathological examination showed inflammatory pseudotumor-like FDCS, immunohistochemical examination showed CD21, CD23, CD35, vimentin (VIM) positive. Patients from 2006 to 2014 repeatedly in the liver, abdominal cavity, chest wall, etc. found in tumor recurrence and metastasis and concurrent resection. At present, patients with stable disease, regular follow-up, no recurrence or transfer to the submission date. Conclusions Liver FDCS is a very rare disease that is diagnosed on the basis of pathological findings. Surgical resection of the tumor is a reliable treatment, the prognosis is good.