钙化性腱膜纤维瘤6例临床病理分析

Clinicopathological features of six cases of calcifying aponeurotic fibroma

目的探讨钙化性腱膜纤维瘤(calcifying aponeurotic fibroma,CAF)的临床病理学特征。方法回顾性分析6例CAF的临床资料、病理学形态及免疫表型并复习相关文献。结果 CAF好发于青少年的手掌与足底,常为缓慢生长的孤立性无痛结节。镜下可见增生的纤维母细胞、散在分布的钙化小灶及软骨小岛。免疫表型:瘤细胞表达vimentin、HHF-35、Calponin、S-100和CD68等。结论 CAF是一种罕见的具有局部侵袭性的软组织肿瘤,结合临床特点、特征性组织学形态可确诊。反复微小创伤及间充质细胞分化可能与CAF的发生相关。

Purpose To investigate the clinicopathologic features of calcifying aponeurotic fibroma（ CAF）. Methods The clinical and pathologic profiles of 6 cases of CAF were retrospectively analyzed. Results CAF appeared a firm,painless and slowly growing mass with a predilection for the distal portions of the extremities in young children and adolescents. Microscopically,the tumor consisted mainly of benign appearing spindle cells,chondroid cells and calcific debris. Immunohistochemically,the tumor cells were positive for vimentin,HHF-35,S-100,Calponin and CD68. Conclusion CAF is a rare,locally aggressive fibroblastic lesion. It can make an accurate diagnosis by integrating the clinical location with histopathological features. Repeated minor trauma to the extremities and some embryologic cell nests maybe its pathogenesis.