颅内恶性孤立性纤维性肿瘤/血管外皮瘤16例临床病理分析

Intracranial malignant solitary fibrous tumor/hemangiopericytoma:a clinicopathologic analyses of 16 cases

目的探讨颅内恶性孤立性纤维性肿瘤/血管外皮瘤(solitary fibrous tumor/hemangiopericytoma,SFT/HPC)的临床病理学特征、诊断、鉴别诊断及预后。方法收集16例颅内恶性SFT/HPC,回顾性分析其临床及影像学资料、组织形态、免疫表型并复习相关文献。结果 16例患者中男性8例,女性8例;年龄31~71岁,平均51岁。镜下肿瘤细胞丰富稍具异性,核分裂象≥5/10 HPF,肿瘤的组织学结构呈条索状、席纹状或无结构状,部分呈经典的SFT样结构,部分呈HPC样结构。免疫表型:93.75%弥漫表达STAT6;CD34阳性率为87.5%,仅6例为弥漫膜阳性。15例随访1~64个月,7例复发。结论恶性SFT/HPC具有恶性的生物学行为,STAT6是恶性SFT/HPC的一种比较特异的生物学标志物,患者的预后与肿瘤的切除范围及长期的随访相关。

Purpose To investigate the clinicopathologic characteristics,diagnosis,differential diagnosis and prognosis of malignant solitary fibrous tumor/hemangiopericytoma（SFT/HPC）.Methods Sixteen cases of intracranial malignant SFT/HPC were retrospectively studied.The clinical data,imaging features,histopathological and immunohistochemical characteristics were analyzed.Results The 8 male and 8 female patients were between 31 and 71 years of age（mean 51）.The median age was 51 years（range,31-71 years）.16 malignant SFT/HPC cases were originated from intracalvarium.The imaging features showed intracranial neoplasms with relatively clear surrounding boundaries.Microscopically spindle shaped cells were hypercellular,and exhibited ≥5 mitoses per 10 HPF.Cytological atypia was mild.The clinicopathologic characteristics included patternless growth pattern,storiform or fascicular growth pattern,solitary fibrous tumor-like regions and hemangiopericytoma-like regions.Tnere were 2 cases with abundant papillary structure and2 with sarcomatous structure,2 with focal necrosis,2 with invaded cerebral tissues,and 10 with invaded meninges.Immunohistochemically,93.75%（15/16） cases were positive for STAT6,with 15/16 showing diffuse staining.87.5%（14/16）cases were positive for CD34,with 37.5%（6/16） showing diffuse staining.81.25%（13/16） cases were positive for BCL-2.68.75%（11/16） cases were positive for CD99.The Ki-67 index ranged from 5% to 40%.Sixteen patients were followed up for 1-64 months,and 7 patients（43.75%） had recurrences.Conclusion Malignant SFT/HPC shares malignant behaviours.STAT6 is a specific marker for the diagnosis of this tumor.The prognosis of malignant SFT/HPC is related to the extent of tumor excision and long-term follow-up.