眼眶成人型黄色肉芽肿的临床分析

Clinical analysis of adult onset orbital xanthogranuloma

目的探讨成人型黄色肉芽肿的临床、影像学和病理学特征。方法回顾性系列病例研究。对天津医科大学眼科医院2015至2017年4例成人型黄色肉芽肿患者的病程和体征、影像学检查、组织病理学检查等临床及随访资料进行分析。结果4例患者中男性1例,女性3例,年龄46~73岁,平均55岁。眼睑肿胀的病程最短6个月,最长6年。1例为双眼患病,3例为单眼患病。4例(5只眼)表现为眼睑肿胀逐渐加重,4例(5只眼)可触及皮下条索状结节、活动度差、无压痛,2例(2只眼)眼周皮肤隆起发黄。4例(5只眼)患者CT检查显示5只眼均可见眼睑肿胀、软组织增厚;3只眼泪腺肿大,3只眼上直肌受累,1只眼外直肌受累,1只眼下直肌受累,1只眼上斜肌受累,其中1只眼上直肌和下直肌同时受累。1例(1只眼)MRI检查显示病变组织在T1加权像、T2加权像均呈中、低信号;3例(4只眼)行彩色多普勒超声检查显示4只眼均可探及眼睑处形态不规则中强回声区,内回声不均匀,局部有低回声,边界欠清;其中1只眼可见血流信号。所有患者均行局部肿物切除术,术后病理学检查组织中均可见泡沫状组织细胞、Touton细胞、淋巴细胞及浆细胞浸润;免疫组织化学染色结果显示3例(4只眼)肿物组织表达CD68、1例(1只眼)表达S-100。术后随访6个月至2年,3例患者未见复发,1例患者术后6个月出现眼部不适,给予口服曲安西龙治疗后症状缓解。结论成人型黄色肉芽肿主要表现为眼睑肿胀、眶周肿物和皮肤颜色的改变,一般无全身受累情况。影像学检查主要表现为眼睑周围软组织、泪腺和肌肉的受累;组织病理学表现的主要特征为泡沫状组织细胞、Touton巨细胞、淋巴细胞和浆细胞浸润。

Objective To investigate the clinical,imaging and pathological features of adult onset xanthogranuloma.Methods Retrospective case series study.The clinical data of 4 adult patients with adult onset xanthogranuloma treated in the Tianjin Medical University Eye Hospital from 2015 to 2017 were analyzed.Results There were 1 male and 3 females,aged 46-73 years,with an average age of 55 years.The duration of eyelid swelling was from 6 months to 6 years.One case was binocular and 3 cases were monocular.There were progressive enlargement of eyelid swelling and subcutaneous cord nodules with poor mobility and no tenderness in all 5 eyes.The eyelid skin in 2 patients (2 eyes) was bulging and yellow.CT examination showed swelling of the eyelids and soft tissue thickening in 5 eyes.There were lacrimal gland enlargement in 3 eyes,upper rectus muscle involvement in 3 eyes,external rectus muscle involvement in 1 eye,lower rectus muscle involvement in 1 eye,superior oblique muscle involvement in 1 eye,and involvement of both the superior and inferior rectus muscles in 1 eye.In 1 patient (1 eye),the lesion showed low and medium signals on T1 weighted images and T2 weighted images.Three patients (4 eyes) underwent color Doppler ultrasonography,showing the echo zone in the irregularly shaped eyelid was strong,the internal echo was uneven,the local echo was low,and the boundary was not clear.One of the eyes showed a blood flow signal.All patients underwent surgical resection.The pathological results showed infiltration of foamy tissue cells,Touton cells,lymphocytes and plasma cells.Immunohistochemistry demonstrated 3 patients (4 eyes) expressed CD68,and 1 patient (1 eye) expressed S-100.After 6 months to 2 years of follow-up,3 patients had no recurrence.One patient developed eye discomfort at 6 months after surgery,and the symptoms were relieved after treatment with triamcinolone.Conclusions Adult onset xanthogranuloma mainly manifest as eyelid swelling,periorbital mass and skin color changes,generally with no systemic involvement.Imaging examination mainly shows the involvement of soft tissues,lacrimal glands and muscles around the eyelids.The main features of the pathology are infiltration of foamy tissue cells,Touton giant cells,lymphocytes and plasma cells.