摘要
目的探讨脂肪纤维瘤病(LPF)的临床病理学特征、诊断与鉴别诊断。方法分析6例脂肪纤维瘤病临床资料、组织形态学特征、免疫组化结果,并结合相关文献进行讨论。结果 6例患儿中男性5例,女性1例。临床均表现为生长缓慢的无压痛性肿块。本文3例为出生不久就发现肿块,另2例为1岁,1例为3岁。镜下肿瘤主要由纤维母细胞和成熟脂肪组织构成。免疫组化:6例vimentin均(+),3例bcl-2和CD99(+),2例CD34、SMA和S-100(+),1例CD68和β-catenin(+);6例desmin、CK、caldesmon和MSA均(-)。结论脂肪纤维瘤病是一种罕见的软组织肿瘤,复发率高,诊断主要依靠病理组织学形态和免疫组化标记。
Objective To study the clinicopathologic features,diagnosis and differential diagnosis of lipofibromatosis.Methods The clinical data,histology,immunohistochemistry of six cases of lipofibromatosis were analysed and discussed with associated literature. Results Six cases of lipofibromatosis included 5 male and 1 female,of whom three patients were found a lump after born soon,two at one year old and one at three years old. The main clinical manifestations were slow-growing local mass with no tenderness. Histologically,the tumor was mainly composed of mature adipose tissue and fibroblasts. Immunohistochemically,of the six cases of lipofibromatosis,the tumor cells both showed diffuse positivity for Vimentin,3 cases showed positivity for Bcl-2 and CD99,2 cases revealed expression of CD34,SMA and S-100,and 1 case showed diffuse positivity for CD68 and β-Catenin,while no expression of Desmin,CK,Caldesmon or MSA presented.Conclusion The lipofibromatosis is a rare soft tissue tumor,with a high risk of local recurrence but no distent metastasis. The accurate diagnosis mainly depends on the pathological histology and immunohistochemical markers.
出处
《诊断病理学杂志》
2018年第2期133-136,共4页
Chinese Journal of Diagnostic Pathology
关键词
脂肪纤维瘤病
婴幼儿
临床病理
免疫组化
Lipofibromatosis
Infant
Clinicopathology
Immunohistochemistry
