10例幼年性黄色肉芽肿临床病理分析

Juvenile xanthogranuloma:clinical and pathological analyses of ten cases

目的探讨幼年性黄色肉芽肿(juvenile xanthogranuloma,JXG)的临床表病理特征。方法分析10例JXG的临床病理及免疫表型特点,并进行文献复习。结果本组男性7例,女性3例,平均年龄(3.88±4.27)岁。8例临床表现为皮肤单发的黄棕色丘疹、结节;1例表现为间断少量阴道流血20天;1例表现为唾液腺多发的结节。镜下瘤细胞位于真皮内,包括早期片状增生的单核组织细胞及中晚期典型的Touton巨细胞,间质纤维化,并伴有多少不等的炎细胞浸润。免疫组织化学染色提示瘤细胞CD163、CD68均阳性,不表达S-100、CD1α、Langerin。结论JXG是一种十分罕见的良性、可自愈的非朗格汉斯细胞组织细胞增生症,临床表现缺乏特异性,组织病理诊断难度较大。CD163较CD68具有更高的敏感性;穿刺病理诊断可避免部分系统型JXG患者进行不必要的手术治疗。

Objective To investigate the clinicopathological features of juvenile xanthogranuloma(JXG).Methods The clinicopathological and immunohistochemical features of 10 JXG cases were analyzed.And related literatures were reviewed.Results The patients were 7 males and 3 females,with average age of 3.88±4.27 years.8 cases presented with single yellow-brown papules and nodules on the skin,1 case presented with intermittent vaginal bleeding for 20 days and 1 case presented with multiple nodules in salivary glands.Microscopically,the tumor cells were in the dermis,including early plaque-proliferated monocytes and typical Touton giant cells in the middle and late stages.Stroma was fibrous,with more or less inflammatory cells infiltration.Immunohistochemically,tumor cells were positive for CD163 and CD68 and negative for S-100,CD1αand Langerin.Conclusion JXG is very rare,and it is a benign and self-healing non-Langerhans cell histiocytosis.Due to the lack of specific clinical features,it is difficult to make precise histopathological diagnosis.CD163 has higher sensitivity than CD68.Puncture pathological diagnosis can prevent part of systemic JXG from unnecessary surgical treatment.