硬化性上皮样纤维肉瘤(SEF)是一种罕见的侵袭性成纤维细胞肿瘤,常见于下肢和肢带深部软组织,多发于中年人,男女发病均等。SEF临床病程呈侵袭性,﹥50%的病例出现局部复发和远处转移。经典SEF可见透明或红染胞质的上皮样细胞呈巢状和索状排列,分布于致密硬化的间质背景中。SEF病例偶见少细胞的束状结构和黏液样区域。 Sclerotic epithelioid fibrosarcoma (SEF) is a rare invasive fibroblastic tumor, common in lower limbs and limb deep soft tissue, occur in middle-aged, equal incidence of men and women. SEF clinical course was invasive,> 50% of the cases of local recurrence and distant metastasis. The classic SEF shows clear or erythroid cytoplasm of epithelial-like cells nested and cord-like arrangement distributed in the dense sclerosis of the interstitial background. Occasionally few cases of SEF bundles of cellular structure and myxoid area.