成人眼眶坏死性黄色肉芽肿的临床病理分析

Necrobiotic xanthogranuloma around the periorbital region in adult: a clinicopathologic analysis

目的探讨成人眼眶渐进性坏死性黄色肉芽肿(necrobiotic xanthogranuloma,NXG)的临床病理学特征、诊断及鉴别诊断。方法回顾性分析4例NXG的临床病理资料,行组织形态学观察和免疫组化检测及随访,并复习相关文献。结果4例患者中男女性各2例,年龄41~59岁,平均47.5岁;临床多为眼眶周围渐进性增大肿物十余年。光镜下均表现为组织细胞增生性病变,真皮内弥漫浸润的肉芽肿性病变及多灶性渐进性坏死交替分布,并可延伸至皮下组织。浸润性肉芽肿由类上皮细胞和泡沫样组织细胞及较多的多核巨细胞组成,其中多为Touton巨细胞。间质内淋巴细胞和浆细胞浸润,伴不同程度的纤维化。例3可见胆固醇结晶沉着和脂质空泡。实验室检查4例患者均存在不同程度的贫血、白细胞减少及血沉增快。仅例1伴血清IgA及IgG升高,其余3例临床均未行免疫球蛋白检查。免疫表型:单核组织细胞及多核巨细胞中KP1和PGM1均阳性。临床治疗均采用局部手术改善症状,术前及术后辅以糖皮质激素治疗。随访8~46个月(平均30个月),4例患者均出现不同程度的复发,目前尚未发现全身系统性并发症。结论NXG是一类具有独特组织学特征的非朗格汉斯组织细胞增生性病变,临床治疗方法有限,且收效甚微;需临床与病理相结合才能做出准确诊断。

Purpose To study the clinicopathologic features,diagnosis and differential diagnosis of necrobiotic xanthogranuloma(NXG)around the periorbital region in adult.Methods The clinical,pathological and immunohistochemical features were evaluated in 4 cases of NXG,as well as follow-up was made.Results Four cases of NXG were retrieved from the pathological archive of this hospital,which were diagnosed and reviewed by two independent pathologists.Their ages ranged from 41 to 59 years(median 47.5 years)with equal gender distribution.Clinically,the majority of patients presented with gradually enlarging,yellowish lesions around the periorbital region for more than 10 years.Histologically,all the NXG cases were characterized by an inflammatory infiltrate,and mainly composed of granulomatous inflammation and necrobiosis in the dermis and subcutaneous tissue.Granulomas consisted of epithelioid cells,foamy histiocytes,foreign body-type multinucleated giant cells,and Touton-type giant cells.Lymphocytes and plasma cells often could be seen with variable degree of fibrosis.Cholesterol crystal and lipid vacuolation were observed in Case 3.On laboratory examination,all 4 patients had different degree of anemia,leukocytopenia and erythrocyte sedimentation rate increase.And the increase of IgA and IgG level in serum was detected in Case 1,but the others were not done clinically.Immunohistochemically,all mononuclear and multinucleated histiocytic cells were positive for KP1 and PGM1.The clinical treatments included surgical resection,supplemented with glucocorticoid before and after surgery.Completed clinical follow-up information was available on all 4 patients,ranging from 8 to 46 months(median 30 months),of which each case showed the evidence of recurrence after clinical treatment.Furthermore,they had not yet been discovered any systemic complications until the end of follow-up.Conclusion NXG is an uncommon non-Langerhans cell histiocytosis,on which appropriate treatments had few and curative effect.The close link of clinic and pathology is helpful for adequate diagnosis of NXG.