11例孤立性纤维性肿瘤的临床病理分析

Clinicopathological analysis of 11 cases of solitary fibrous tumor

目的 探讨孤立性纤维性肿瘤(SFT)的临床病理、免疫表型和预后。方法 收集11例SFT患者的临床及病理资料,行HE染色及免疫组化检测,总结其临床及影像学表现、组织学形态、免疫表型特征及预后,并结合相关文献进行复习。结果 11例患者中,男性9例,女性2例,年龄25~85岁。影像学均表现为结节状软组织肿块。肿瘤最大径3.5~30 cm,切面实性,部分有完整包膜。肿瘤细胞呈卵圆形或梭形、短梭形,疏密不均,可见薄壁鹿角形血管。部分病例细胞轻-中度异型,偶见奇异形核。免疫组化结果显示,所有肿瘤均表达CD34,8例STAT6核阳性。目前手术完整切除仍是SFT的首选治疗方法。本组10例患者获得随访,其中8例无病生存,1例死亡患者原因不详,1例术后复发仍带瘤生存。结论 SFT具有相对独特的病理组织学和分子遗传学改变,发生于胸膜外者生物学行为更具侵袭性。具有恶性组织学特征者具有较高的局部复发和转移率,因此正确的诊断对于治疗和患者管理具有重要意义。

Objective Objective To investigate the clinicopathology,immunophenotype and prognosis of solitary fibrous tumor(SFT).Methods The clinical and pathological data of 11 patients with SFT were collected.HE and immunohistochemical staining were performed,the clinical and imaging manifestations,histological morphology,immunophenotypic characteristics,and prognosis were summarized,and the relevant literatures were reviewed.Results There were 11 cases of SFT,including 9 males and 2 females.The range of onset age was 25-85.Imaging findings all present as nodular soft tissue masses.Gross observation showed that the maximum diameter of the tumor was 3.5-30 cm.The section was solid with or without complete capsule.The tumor cells was typically oval,spindle or fusiform.The cell density varied from sparse to dense with characteristic staghorn-like vessels.In some cases,the tumor cells have mild-to-moderate atypia with occasionally bizarre morphology.All tumors cells was CD34 positive,and STAT6 was positive in 8 cases.Surgical complete resection was still the first treatment method for SFT.Among the 10 patients in this group who received follow-up result,8 survived disease-free,1 died due to unknown causes,and 1 survived with tumor after surgery.Conclusion SFT has a relatively unique histological morphology and molecular genetic changes.Extrapleural SFT has more invasive biological behavior.Patients with malignant histological features were directly diagnosed as malignant SFT,with a high rate of local recurrence and metastasis.Therefore,correct diagnosis is of great significance for treatment and clinical management.