腹腔孤立性纤维瘤18例临床分析

Clinical characteristics of abdominal solitary fibrous tumor： an analysis of 18 cases

目的总结腹腔孤立性纤维瘤的临床表现、病理特点及诊疗方法。方法回顾性分析2000年1月至2016年12月北京协和医院收治18例腹腔孤立性纤维瘤病例，采集并分析其基本信息、临床表现、血清学检查、影像学检查、手术方式和病理特点。结果14例以腹部肿块为首发症状。所有患者血常规、肝肾功能、凝血正常，4例患者有肿瘤标志物的异常。腹腔孤立性纤维瘤在B超下多为形态规则的低回声肿块，CT下表现各异。本组18例均行手术治疗，经术后病理学诊断均证实为孤立性纤维瘤。腹腔孤立性纤维瘤的大体表现各异，免疫组化以Ki-67、SMA、CD34（血管）、S-100阳性的比例最高。结论腹腔孤立性纤维瘤早期诊断较困难，术前诊断率低，病理诊断尚缺乏特异性标志物，彻底切除肿瘤是目前唯一可靠的根治方法。

Objective To summarize the clinical manifestations, pathological features, diagnosis, and treatment of abdominal solitary fibrous tumor （SFT）. Methods The data of clinical manifestations, pathological features, diagnosis, and treatment of 18 patients with abdominal SFT admitted to Department of General Surgery, Peking Union Medical College Hospital between January 2000 and December 2016 were retrospectively reviewed. Results Abdominal mass was first presented in 14 patients. The blood routine, liver, kidney, and coagulation function were normal in all the patients. Tumor markers were abnormal in 4 cases. Most SFT showed hypoechoic mass with regular shape under ultrasound. CT findings differed among the cases. All the 18 patients underwent surgical treatment, and the diagnosis was proved by the postoperative histopathology. SFT differed in general morphology. The positive rate of Ki-67, smooth muscle actin （SMA）, CD34, and S-100 was high under immunohistochemistry. Conclusions Early diagnosis of abdominal SFT is difficult, leading to a low preoperative diagnosis rate. Complete removal of the tumor is the only reliable method of radical treatment.