骨促结缔组织增生性纤维瘤7例临床病理分析

Desmoplastic fibroma of the bone: a clinicopathological analysis of seven cases

目的探讨骨促结缔组织增生性纤维瘤(desmoplastic fibroma,DF)的临床病理学特征。方法回顾性分析7例骨DF的临床资料、病理学形态及免疫表型特征等并复习相关文献。结果骨DF好发于青少年,可累及任何骨,长管状骨及下颌骨好发,表现为境界清晰的溶骨性病变且可累及周围软组织,具有良性的形态改变:增生的梭形纤维母细胞及交错的胶原纤维。免疫表型:瘤组织表达vimentin。结论骨DF是一种罕见的形态学呈良性改变,而生物学行为呈侵袭性的骨肿瘤,可复发,结合临床特点,掌握其特征性的组织学形态可作出正确诊断。外伤、体内激素、染色体畸变可能与骨DF的发生相关。

Purpose To investigate the clinicopathologic features of desmoplastic fibroma of bone （DF）. Methods The clinical and pathologic profiles of 7 cases were retrospectively analyzed. Results Desmoplastic fibroma of bone was a rare neoplasm of bone, which typically occurred in young children and adolescents. The tumor showed infiltrative and locally aggressive nature, the most common sites were long tubular bones and mandible. Microscopically, the tumor was composed of sparse fibroblasts in a rich background of collagen fibers. Immunohistochemically, the tumor cells were positive for vimentin and SMA. Conclusion Desmoplastic fibroma is a rare, locally aggressive fibroblasticlesion of bone. It can make an accurate diagnosis by integrating the clinical location with histopathological features. Trauma, hormone and horomosome aberration may be related with its pathogenesis.