孤立性纤维性肿瘤临床病理分析及风险评估

Clinical pathological analysis and risk assessment analysis of solitary fibrous tumor

目的孤立性纤维性肿瘤(solitary fibrous tumor,SFT)作为间叶来源的梭形细胞肿瘤,病理特征复杂,临床易误诊,肿瘤生物学行为较难预测。本研究旨在通过16例全身多部位发生的SFT探讨其临床病理特征、诊断与鉴别诊断、肿瘤风险等级的评估与预后。方法收集2007-05-18-2019-01-22首都医科大学附属北京安贞医院诊治的16例SFT患者的临床病理资料,回顾性分析其病理组织学特点及免疫组化表达,并结合文献及随访资料进行分析。结果16例SFT患者的年龄为12~71岁,中位年龄为59.5岁。肿瘤部位包括胸膜(3例)、肺(6例)、左心房(1例)、心包(1例)、腹膜后(1例)、头颈及四肢(4例)。临床主要表现为局部肿块及相应的压迫症状。镜下观察肿瘤形态多样,梭形或圆形细胞呈无结构的生长模式,细胞疏密分布,细胞间伴有粗大的玻璃样变性胶原纤维及血管外皮瘤样结构。6例恶性SFT细胞密度增加,细胞异形显著,核分裂象≥5个/10HPF,伴有出血及坏死。免疫表型CD34、CD99和Bcl-2表达情况各不相同,其中STAT6阳性率为100%(16/16),CD34阳性率为87.5%(14/16)。结论肿瘤具有特征性形态结构,有时术中冰冻诊断易误诊,免疫组化特异性标志物STAT6具有重要诊断价值。肿瘤风险等级不能完全反映预后。

OBJECTIVE Solitary fibrous tumor(SFT)is a spindle cell tumor of mesenchymal origin.Its pathological features are very complicated and it is easy to be misdiagnosed in clinic,especially its biological behavior is difficult to predict,the purpose of this study was to investigate the relationship between clinicopathological features,diagnosis and differential diagnosis,tumor risk grade and prognosis in 16 cases of multiple site SFT.METHODS The clinical and pathological data of 16 patients with SFT diagnosed and treated in Beijing Anzhen Hospital Affiliated Capital Medical University from May 18,2007 to January 22,2019 were retrospectively analyzed for histopathological characteristics and immunohistochemical expression,combined with literature and follow-up Data analysis discussion.RESULTS The age of 16 patients with SFT ranged from 12 to 71 years old,and the median was 59.5 years old.The tumor site included pleura(3 cases),lung(6 cases),left atrium(1 case),pericardium(1 case),retroperitoneum(1 case),head and neck,and limbs(4 cases).The main clinical manifestations were local mass and corresponding compression symptoms.Microscopically,the tumor was characterized by a wide variety of morphologies,with no structural growth pattern of spindle or round cells,dense distribution of cells,and presence of large Hyaline collagenous fibers and hemangiopericytoma-like structures.In 6 cases of malignant SFT tumor,the cell density was increased,the cell Atypia was remarkable,the mitotic figure≥5/10 HPF,accompanied by hemorrhage and necrosis.The immunophenotypes of CD34,CD99 and Bcl-2 were different,the positive rate of STAT6 was 100.0%(16/16),the positive rate of CD34 was 87.5%(14/16).CONCLUSIONS The tumor has characteristic morphological structure,sometimes the intraoperative frozen diagnosis is easy to be misdiagnosed,immunohistochemical specific marker STAT6 has important diagnostic value.The tumor risk level does not fully reflect the prognosis.