脂肪纤维瘤病的临床病理与鉴别诊断

Clinicopathological analysis and differential diagnosis of lipofibromatosis

目的探讨脂肪纤维瘤病的临床病理特征、诊断及鉴别诊断。方法研究2019年5月14日福建中医药大学附属泉州市正骨医院诊断的一例脂肪纤维瘤病的临床资料、病理形态学特征、免疫组化结果,并结合相关文献进行讨论。结果男性患儿,2岁1个月,出生后即发现患儿右拇指伸直活动受限,被动活动不能伸直,右手拇指指间关节屈曲畸形,右食指呈半屈曲位畸形,主动伸直受限。术后送检组织镜下纤维细胞样梭形细胞条束状穿插分隔脂肪组织呈小叶状。免疫组化:梭形细胞Vimentin、CD34、CD99阳性,β-catenin、SMA、Actin、Desmin、S100、NF、Bcl-2阴性。结论脂肪纤维瘤病是一种罕见的好发于婴幼儿的软组织肿瘤,易复发,组织形态结构与其他肿瘤有一定相似,依据组织形态学和免疫组化结果可明确诊断。

Objective To explore the clinicopathological features,diagnosis,differential diagnosis,treatment,and prognosis of lipofibromatosis.Methods The clinical data,pathomorphological characteristics,and immunohistochemical results of a case of lipofibromatosis diagnosed by Quanzhou Orthopedic Hospital,Fujian University of Traditional Chinese Medicine on May 14,2019 were analyzed and discussed with associated literature.Results The children(male,2 years and 1 month old now)were found to have limited right thumb extension and passive movement,flexion deformity of interphalangeal joint of right thumb,semi-flexion deformity of right index finger,and limited active extension after birth.Under the microscope,fibrous cells like spindle cells were interspersed to divide adipose tissue into lobules.Immunohistochemical staining showed that spindle cells Vimentin,CD34,CD99 were positive,β-catenin,SMA,Actin,Desmin,S100,NF and Bcl-2 were negative.Conclusion The lipofibromatosis is a rare soft tissue tumor that occurs frequently in infants and is easy to recur.The histopathological structure is similar to other tumors,which can be diagnosed by histomorphology and immunohistochemistry.