Lipofibromatosis in children:a clinicopathologic analysis of 13 cases
LI Xiao-wei
ZHU Xue-ming
WANG Xing-dong
WU Yi
ZHANG Min
Abstract：Objective To explore the clinical and pathological characteristics,diagnosis,and differential diagnosis of childhood lipofibromatosis(LPF).Methods The clinical data,histopathological characteristics,and immunophenotypic Results were analyzed in 13 children with LPF,with review of relevant literature.Results Among the 13 patients,there were 8 males and 5 females.Age ranged from 6 months to 10 years(median age 3 years).The clinical manifestations were painless slow-growing masses in deep tissues.Histologically,the appearance was similar,with more adipose tissue observed,divided by fibroblast and myofibroblast bundles,with 2 cases invading the surrounding skeletal muscles.Immunophenotyping showed that tumor cells expressed vimentin and were positive for CD34,SMA and H3K27me3 to varying degrees,but did not express β-catenin,desmin,S100,NSE,PanTRK,SOX10,and ALK;Ki-67 proliferation index was 1-2%.Conclusion LPF is an intermediate soft tissue tumor that more commonly occurs in infants and young children,with a high recurrence rate and non-specific clinical manifestations.Its histopathological morphology is similar to other tumors,and a clear diagnosis should be made based on immunophenotypes.
Keywords：Adipose fibroadenomaChildhoodPathologyImmunohistochemistryCalcified aponeurosis fibroadenoma
Publication Date：2025-06-28
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 673-676 )