骨成纤维性纤维瘤的临床病理研究

Study of Clinical and Pathological Features of Desmoplastic Fibroma of Bone

目的探讨骨成纤维性纤维瘤（ＤＦ）的临床病理特点、组织发生、临床误诊及复发因素。方法收集１１例骨成纤维性纤维瘤病例，男６例，女５例，年龄１１－４２岁。免疫组织化学ＡＢＣ法标记ＳＭａｃｔｉｎ，Ｄｅｓｍｉｎ，Ｖｉｍｅｎｔｉｎ，ＮＳＥ，Ｓ－１００蛋白，ａ－ＡＴ（ａ－平滑肌肌动蛋白）及Ｌｙｓｏｚｇｍｅ。结果Ｖｉ－ｍｅｎｔｉｎ阳性１１例，Ｄｅｓｍｉｎ阳性９例、弱阳性２例，ＳＭａｃｔｉｎ阳性１０例、弱阳性１例，ＮＳＥ、Ｓ－１００蛋白、ａ－ＡＴ及Ｌｙｓｏａｇｍｏ均阴性。３例复发，４例病理性骨折，１１例术前无一例诊断ＤＦ。结论ＤＦ含肌纤维母细胞，此种细胞在组织学上具有平滑肌和纤维母细胞特征。造成临床误诊与复发的主要原因是缺乏特征性的临床表现及Ｘ线表现，肿瘤无包膜。作者发现瘤细胞具有沿骨小梁及哈佛管侵袭性生长的生物学行为，与肿瘤复发有直接相关性。

Objective The pathological features of desmoplastic fibroma (DF) in bone and itspathogenesis, recurrence as well as the cause of misdiagnosis were analysed. Methods Eleven cases of DFwere studied, including 6 females and 5 males, with a range of 11 to 42 years. Immunohistochemical studieshave been used to evaluate the patterns of immunoreactivity of DF. Results lmmunohistochemical stains ofDF showed that 11 cases were positive for Vimentin, 9 cases were positive for Desmin (2 cases were slightpositive), 10 cases were positive for SMactin (1 case is slight positive). All were negative for NSE, S-100protein, a - AT, lysozyme. Three cases recured, 4 cases occured pathological fracture. Eleven cases DF werenot diagnosed before operation. Conclusions DF contains myofibroblasts, featuring both smooth musclecells and fibroblasts. Radiologically and clinically, DF is a typical, which is liable to recur and to be mis-diagnosed. The tumor without capsule invades along bone trabecula and Haversian system. As a result, theaggressive behavior of this tumor was closely related to its recurrence.