摘要
目的探讨骨原发性促结缔组织增生性纤维瘤（desmoplastic fibroma,DF）的临床病理特征。方法分析8例骨原发性DF患者的临床表现、影像学改变、病理形态、免疫表型和随访结果,并复习相关文献。结果 8例DF中女性3例,男性5例,年龄18~56岁,平均30.5岁。发生部位：桡骨2例,跟骨、尺骨、趾骨、椎体、上颌骨、髋臼各1例。临床表现为病变部位的疼痛和肿胀。影像学表现为溶骨性占位,部分患者骨质破坏区内有粗大的骨小梁形成。大体肿瘤边界不清,灰白色无光泽,质地韧。镜下见肿瘤由纤细的梭形细胞和胶原纤维组成,瘤细胞呈束状、平行状或波浪状排列,细胞无异型,核分裂象罕见,肿瘤边界不清,呈浸润性生长,瘤细胞在骨小梁间穿插,有些病例侵犯骨皮质或继发动脉瘤样骨囊肿;1例复发后局部恶变,恶变成分为高分化纤维肉瘤,恶变病例细胞稍丰富,生长活跃,核有异型,可见核分裂象。免疫表型：瘤细胞胞核表达β-catenin,vimentin弥漫阳性;部分细胞表达SMA,Ki-67增殖指数﹤5%;MDM2、desmin、CD34和S-100均阴性。结论骨原发性DF罕见,诊断主要依赖影像学、病理形态学和免疫表型的结合,需与髓内高分化骨肉瘤、骨高分化纤维肉瘤、骨神经纤维瘤、骨神经鞘瘤、骨平滑肌瘤、纤维结构不良等良恶性病变鉴别。DF预后好,但易复发,少数病例可出现肉瘤样变,骨原发性DF被定性为中间型纤维源性肿瘤。
Purpose To investigate the clinical pathological features of primary bone desmoplastic fibroma（ DF）. Methods To analyze the clinical manifestation,radiological changes,pathological features,immunophenotype and follow-up in eight cases of primary bone DF and reviewed the literature. Results 8 patients,3 females and 5 males,ranged in age from 18 to 56 years with the mean age of 30. 5 years. Involvement of bones included radius（ 2 cases）,ulna,mandible,ilium,calcaneus,vertebra and phalanx. Pain and swelling of involved bone were the usual presenting symptoms. Radiologically,these tumors were usually lytic,destructive,and had coarsely large trabeculared bone formation in some patients. Macroscopically,the cut surface of DF showed a gray-white with gritty texture and poor-defined margins. Histologically,the tumor was composed of spindle tumor cells and collagen fibers. The spindle-shaped tumor cells were arranged in fascicles,parallel-like or interlacing bundles that permeated surrounding cortical and / or cancellous bone.They were characterized by little cytological atypia and few mitotic figures. The tumor was ill-defined and grew invasively,and occasionally bone cortex was involved or secondary aneurysmal bone cyst was formed. One patient recurred and then progressed to well-differentiated fibrosarcoma,in which the cells were a little abundant and grew actively,and showed a minimal cytological atypia and relatively few mitosis. Immunohistochemically,the tumor cells diffusely expressed β-catenin in the nuclei and vimentin both in nuclei and cytoplasm,and focal expressed SMA. And less than 5% cells expressed Ki-67. But it was negative for MDM2,desmin,CD34 and S-100. Conclusions Primary bone DF is rare,and its diagnosis depends on the combination of radiographic changes,pathological morphology and immunophenotype. It should distinguish it from low-grade central osteosarcoma,well-differentiated fibrosarcoma,neurofibromatosis,neurinoma,liomyoma of bone,fibrous dysplasia and so on. The prognosis of DF is excellent,however,it easily recurs,and few cases may become sarcomatous. Primary bone DF is thus defined as a borderline fibrous tumor.
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2015年第12期1370-1373,共4页
Chinese Journal of Clinical and Experimental Pathology
