Congenital juvenile xanthogranuloma:A clinicopathological analysis of eight cases
XIAO Yao
WU Baoyu
WANG Han
LI Jingyu
MA Zhihui
Abstract：Objective To explore the clinicopathological features,diagnosis,differential diagnosis,treatment and prognosis of congenital juvenile xanthogranuloma(JXG).Methods The clinical data of 8 cases of congenital JXG were collected.Hematoxylin-eosin(HE)staining and immunohistochemical EnVision two-step staining were used to analyze the clinicopathological features,and the relevant literature was reviewed.Results In this group,there were 6 male cases and 2 female.Seven cases presented with single red or yellow nodules on the skin,with a diameter ranging from 0.5 to 4.0 cm.One case had multiple small nodules with a diameter of 1.0-2.0 cm.Microscopic examination revealed diffusely proliferating mononuclear histiocytes.The cytoplasm was abundant and eosinophilic.Variable numbers of foam cells and Touton cells were observed,accompanied by infiltration of eosinophils and lymphocytes to varying degrees.Immunohistochemical staining showed that the tumor cells were positive for CD68 and CD163,but negative for S-100,Langerin,CD1a,and ALK.The pathological diagnosis was juvenile xanthogranuloma.Conclusion Congenital juvenile xanthogranuloma is relatively rare.Microscopically,it is characterized by histiocyte proliferation with varying numbers of Touton cells.Familiarity with the clinicopathological features of this disease can avoid misdiagnosis and overtreatment.
Keywords：Juvenile xanthogranulomaCongenitalGiant typePathological diagnosis Immunohistochemistry
Publication Date：2025-11-28
Online Publishing Date：2025-12-04(First online date of this platform, not the publication date of the document)
Pages：4( 1457-1460 )