四肢孤立性纤维瘤的诊断及外科治疗

Diagnosis and surgical treatment for solitary fibrous tumor limbs

目的探讨四肢孤立性纤维瘤(SFT)的临床表现、影像学特点及病理学特征、手术治疗方式。方法回顾性分析中山大学孙逸仙纪念医院关节外科9例经病理确诊的四肢孤立性纤维瘤患者的临床表现、影像学特点及病理学特征。9例患者中3例男性,6例女性,中位数年龄44岁(15～55岁);均进行MRI检查,手术切除后送病检行免疫组化,比较其临床表现、影像学特征及病理学特征。结果9例患者中,肿瘤分布于上肢者6例,下肢3例。患者首要临床症状均为无痛性肿块,其中3例出现患肢远端麻木等压迫症状。影像学方面,9例患者术前行MR检查,了解其边界及邻近关系,7例患者MR表现为T1加权像为等信号,2例为低信号; T2加权像4例为混杂高信号,1例为高信号; 9例患者增强像为不均匀明显强化;边界清楚者6例,模糊者3例。免疫组化结果显示9例患者中波形蛋白(vimentin)均为阳性(9/9),跨膜糖蛋白分子CD34阳性者8例(8/9); B淋巴细胞瘤-2因子(bcl-2)阳性者6例(6/9),信号转导与转录激活因子-6(STAT-6)阳性者3例(3/9);增殖细胞相关核抗原Ki67分布于5%～65%。结论影像学中MRI通过不同加权像可以对SFT的瘤体边界进行定位,确定肿瘤的邻近关系,为SFT的诊断提供依据,为手术方案的选择提供依据。进行病理学免疫组化是诊断孤立性纤维瘤的主要手段,并可指导术后辅助放化疗方案的制定。

Objective To investigate the clinical feature s of solitary fibrous tumor(SFT) in limbs and the optimal surgical approaches effect. Methods The clinical records of nine patients with SFT in limbs were retrospectively reviewed in department of orthopaedics of Sun Yat-sen Memorial Hospital between January2005 and December 2016. There were three males and six females,with a median age of 44 years(15 ~ 55years). Laboratory examinations showed normal results. MRI and biopsy were performed in all the cases. All the patients underwent surgical treatment with resection performed. The tumors originated from up libms in six patients,from lower limbs in three patients. Results All the tumors were totally excised. There was no postoperative mortality nor major complications. Among the nine patients,six patients had tumors in the upper extremities and three in the lower extremities. The primary clinical symptoms were painless masses,three of them with the distal limb numbness symptoms of compression. All the patients accepted MRI examinations to analyse their connections,confirm the borderline and size of the diseased tissues and its relation to the adjacent tissues. MRI examinations demonstrated that appearances of lesions were various. The expressions of vimentin(nine cases),CD348(eight cases),bcl-2(six cases),signal transducers and activators of transcription(STAT)-6(three cases) and Ki67(5% to 65%) were positive by immunohistochemical analysis. Conclusions MRI canbe used to survey the tumor boundary of SFT,determine the adjacent relationship of SFT,provide the proofs for the diagnosis of SFT,and provide the basis for the selection of surgical procedures. Pathological immunohistochemistry is the main method for the diagnosis of solitary fibroma and can guide the formulation of adjuvant radiotherapy and chemotherapy after operation.