幼年性黄色肉芽肿13例病理学分析

Pathological analysis of 13 cases of juvenile xanthogranuloma

目的总结幼年性黄色肉芽肿的病理学特点。方法回顾性分析新乡医学院第一附属医院2018年1月至2019年6月确诊的13例幼年性黄色肉芽肿患者的组织病理学特点。结果13例患者中,男8例,女5例,发病中位年龄为1岁(2个月~11岁),11例为单发皮损,1例为多发皮损,另有1例发生于颅内。其中单发及多发皮损者病变多数位于头面部,未见皮肤以外器官受累;颅内病变为单发、无皮肤及其他器官系统受累。组织病理学表现为表皮轻度变薄,真皮内见边界相对清楚、无包膜的细胞团。高倍镜下可见病变由不同比例的组织细胞、Touton巨细胞及多种炎症细胞组成。免疫组织化学结果显示,皮损处浸润细胞中CD68、CD163、CD4、CD43均呈阳性表达,CD1a呈阴性表达;多数细胞中S-100呈阴性表达,仅少数病例浸润细胞中S-100呈阳性表达。结论幼年性黄色肉芽肿多为单发皮损,表皮无受累或略变薄,真皮内可见组织细胞浸润,形态相对单一,可见Touton巨细胞及炎症细胞;不典型者及其他器官系统病变可表现为梭形细胞或圆形、类圆形淋巴样细胞浸润,阳性表达CD163、CD68、CD4及CD43。

Objective To summarize the pathological characteristics of juvenile xanthogranuloma.Methods The histopathological characteristics of 13 cases of juvenile xanthogranulomatosis diagnosed in the First Affiliated Hospital of Xinxiang Medical University from January 2018 to June 2019 were analyzed retrospectively.Results Among the 13 patients,there were 8 male patients and 5 female patients;the median age of onset was 1 year old(2 months to 11 years old).There were11 cases with single lesions,1 case with multiple lesions and 1 case with central nervous system lesion.Most of single and multiple lesions were located in the head and face,and no other organs except for the skin were involved.Histopathological findings showed that the epidermis was slightly thinned,and the dermis showed relatively clear boundaries and no envelope cell clusters.Under the high power microscope,the lesions were consisted of different proportion of histocytes,Touton giant cells and various inflammatory cells.Immunohistochemical results showed that CD68,CD163,CD4,CD43 were positive expression in infiltrating cells;CD1 a was negative expression;S-100 was negative expression in most cells,and S-100 was sporadically positive expression in some cases.Conclusion Juvenile xanthogranuloma is mostly single skin lesion.The epidermis is not involvedor slightly thinned.The infiltration of histiocytic cells can be seen in the dermis,with relatively single morphology,including Touton giant cells and inflammatory cells.The spindle cells or round,quasi-round lymphoid cells infiltration can be observed in atypical patients or other organ system lesions;and the CD163,CD68,CD4 and CD43 are positive expression in the infiltration cells.