Two Cases of Angiomatoid Fibrous Histiocytoma with Literature Review
ZHANG Jing
LI Yixiao
QI Minxian
JIN Yiping
WANG Lu
XUE Yun
CHAI Yamei
LI Dandan
LI Xuebing
Abstract：Objective To investigate the clinicopathological features and diagnostic points of angiomatoid fibrous histiocytoma(AFH).Methods Immunohistochemical findings and EWSR1 gene fluorescence in situ hybridization results of two AFH cases admitted to Henan Chest Hospital from 2014 to 2024 and 68 AFH cases reported in literatures were collected.Clinical features,pathological subtypes,immunohistochemical profiles and EWSR1 molecular alterations were retrospectively analyzed.Results Among the two local cases,there was one female and one male patient aged 50 years and 13 years respectively.No postoperative recurrence was observed,and both cases were classified as solid subtype.Immunohistochemically,the tumor cells were diffusely positive for EMA,CD68,CD99,Desmin,Vimentin,Leu-7,Bcl-2 and CD31,while negative for CK,CK5/6,MyoD1,Myogenin,HMB45,CD34,S-100,SOX-10 and CD21.The Ki-67 proliferation index was 5％in both cases,and EWSR1 gene split signals were detected in all specimens.For the 68 literature-reported cases,there were 31 females and 37 males with an age range of 1 to 76 years.One patient died,two cases developed tumor recurrence,50 cases had no recurrence after surgery,two cases were lost to follow-up,and 13 cases lacked follow-up data.Pathological subtypes included 11 solid-type cases,5 myxoid-type cases,22 classical-type cases,and 30 cases with undetermined subtypes.Immunohistochemical positive rates were as follows:Vimentin(34/37),CD99(38/46),CD68(36/44),EMA(39/52),Desmin(36/52),α-SMA(24/52),ALK1(3/13),CD31(2/20),CD56(1/12),CD34(1/50)and CK(1/56).S-100(47/47),HMB45(21/21),CD117(11/11)and INI1(10/10)were consistently negative in all tested specimens.The Ki-67 proliferation index ranged from 2％to 20％.EWSR1 molecular testing was performed in 23 cases,including three cases with EWSR1-CREB1 fusion,two cases with EWSR1-ATF fusion,two cases with EWSR1-CREM fusion,and 16 cases with unclassified EWSR1 rearrangement.Conclusion AFH predominantly occurs in the young population,presenting distinctive histological morphology and immunophenotype.The majority of AFH cases harbor EWSR1 gene rearrangement.Comprehensive evaluation combining clinical manifestations,histopathology,immunohistochemistry and molecular detection can effectively improve the diagnostic accuracy of AFH.
Keywords：angiomatoid fibrous histiocytomapathological featuresImmunohistochemistryEWSR1 genediagnosis
Publication Date：2026-07-13
Online Publishing Date：2026-09-12(First online date of this platform, not the publication date of the document)
Pages：5( 2333-2337 )