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目的探讨脂肪纤维瘤病的临床病理特征、诊断与鉴别诊断、治疗及预后。方法对6例脂肪纤维瘤病临床病理特征、组织形态学及免疫组化进行观察分析并复习相关文献。结果 6例患者中4例男性,2例女性,年龄6月~6岁,平均24.8月,表现为发生于手、足、胸壁、腰部、臀部和背部界限不清的缓慢生长的无痛性肿块。肿瘤由纤维性梭形细胞和成熟的脂肪组织构成。免疫组化:梭形细胞表达vimentin(6/6)、CD34(6/6)、bcl-2(4/6)和CD99(5/6),其中2例SMA灶性阳性,6例均不表达desmin、β-catenin、Myogenin和Myo D1。结论脂肪纤维瘤病是一种好发于婴幼儿的软组织肿瘤,较罕见,属于中间型肿瘤,容易与其他肿瘤混淆导致误诊,只要充分认识其临床、组织形态学与免疫组化特征,往往可以对其作出正确诊断。
Objective To investigate the clinicopathological features, diagnosis and differential diagnosis, treatment and prognosis of fatty fibromatosis. Methods The clinical and pathological features, histomorphology and immunohistochemistry of 6 cases of fatty fibromatosis were observed and analyzed and relevant literatures were reviewed. RESULTS: Of the 6 patients, 4 were male and 2 were female. They ranged from 6 months to 6 years (mean 24.8 months) and presented with slowly growing, painless lumps that occurred in the hands, feet, chest wall, waist, buttocks, and back . Tumors consist of fibrous spindle cells and mature adipose tissue. Immunohistochemistry: The spindle cells expressed vimentin (6/6), CD34 (6/6), bcl-2 (4/6) and CD99 (5/6) Expression of desmin, β-catenin, Myogenin and Myo D1. Conclusions FFA is a soft tissue tumor that occurs in infants and young children. It is rare and belongs to the intermediate type. It is easy to be confused with other tumors and leads to misdiagnosis. As long as its clinical, histomorphological and immunohistochemical characteristics are well understood, Make a correct diagnosis of it.