恶性孤立性纤维性肿瘤的临床病理特征分析并文献复习

目的:探讨恶性孤立性纤维性肿瘤的临床病理特征及鉴别诊断。方法:对9例恶性孤立性纤维性肿瘤进行光镜及免疫组织化学研究，并复习相关文献。结果:男性6例，平均年龄为49.7岁;女性3例，平均年龄为48.3岁;平均肿瘤直径为9.39 cm。镜检:细胞稀少区和细胞丰富区交替分布，两者之间有粗的玻璃样变胶原纤维分隔。密集区肿瘤细胞丰富，呈束状、旋涡状排列，可见血管外皮瘤样结构；瘤细胞呈梭形，异型性明显，有肿瘤性坏死，核分裂均大于4个/10 HPF。免疫表型:肿瘤细胞STAT6和Vimentin一致阳性表达，CD34、BCL-2、CD99不同程度阳性表达。结论:恶性孤立性纤维性肿瘤确诊依据形态学及免疫组化，需与形态学表现相似的其他恶性梭形细胞肿瘤鉴别，其预后不良，易复发和转移，根治性切除是主要治疗手段，必要时辅助放化疗。

Clinicopathological analysis of malignant solitary fibrous tumor and literature review

Objective:To investigate the clinicopathologic characteristics,differential diagnosis of malignant solitary fibrous tumor.Methods:Nine cases of MSFT were reported.The clinical features,immunohistochemical analysis and imaging appearance were observed,and sacro-literatures were reviewed.Results:Among the 9 patients,6 were male,and 3 were female.The average age of 49.7 in males and 48.3 in females.The average diameter of the tumor was 9.39 cm.Histologically the tumor was composed of areas of altemating hypercellularity and hypocellularity.In some areas,hemangiopericytoma-like structure was present.The tumor cells were spindleshaped with marked nuclear atypia and increased mitotic activity.Immunohistochemically,STAT6 and Vimentin were always positive,CD34,BCL-2,CD99 were different degree positive.Conclusion:Diagnosis of MSFT mainly relies on morphologic features and immunohistochemistry.Differential diagnosis includes spindle-shaped cellular mesenchymal tumors.The prognosis of MSFT is poor and tends to recur and even metastasize.The major treatment is to completely resect it by operation and long-term clinical follow-up is necessary.