眼眶横纹肌肉瘤的临床特征及病理学分析

Clinical features and pathological analysis of orbital rhabdomyosarcoma

目的分析眼眶横纹肌肉瘤的临床表现、影像学特征、治疗方法及病理特点。方法回顾性分析天津医科大学第二医院眼科1980年至2018年眼眶横纹肌肉瘤36例(36眼)的临床资料。结果患者年龄5个月~14岁,平均(6.54±3.79)岁。眶上象限为其好发部位。就诊的主要症状为眼球突出。B超检查,肿瘤显示为低或无回声区;彩色多普勒探查,肿瘤内可见动脉频谱,血流丰富。28例行CT扫描呈软组织密度影,其中骨质破坏6例。10例行MRI检查,8例T1WI显示为中等或中等偏低信号,T2WI为高信号。36例中眶内容摘除4例,前路开眶切除肿物32例。病理学检查显示:亚型中胚胎型20例(55.6%),腺泡型9例(25.0%),多形型7例(19.4%)。结论眼眶横纹肌肉瘤属高度恶性肿瘤,传统的B超、CT、MRI结合PET-CT等影像学检查可以指导诊断、治疗,监测复发与转移。

Objective To analyze the clinical manifestations,imaging characteristics,treatment and pathological types of orbital rhabdomyosarcoma.Methods The clinical data of 36 eyes of 36 cases of orbital rhabdomyosarcoma from 1980 to 2018 in Second Hospital of Tianjin Medical University were analyzed retrospectively.Results The ages ranged from 5 months to 14 years,with an average of(6.54±3.79)years.The tumors were most commonly located at supraorbital quadrant.Proptosis was the most common initial clinical sign.B-ultrasound examination showed low-echo or echoless areas.The color Doppler ultrasonography exploration showed arterial spectrum and blood flow was abundant.Twenty-eight patients underwent CT scans,the tumors showed soft tissue density,6 cases showing bone destruction.Ten patients underwent MRI examinations,among which 8 cases displayed as iso-or slightly hypo-signal on T1WI,and hyper-signal on T2WI.In all the 36 cases,evisceration of orbital in 4 cases,and tumor was removed through anterior orbitotomy in 32 cases.The pathological examination showed that embryonal rhabdomyosarcoma accounted for 20 cases(55.6%),alveolar rhabdomyosarcoma and pleomorphic rhabdomyosarcoma accounted for 9 cases(25.0%)and 7 cases(19.4%)respectively.Conclusion Orbital rhabdomyosarcoma is a highly malignant tumor.Traditional B-ultrasound,CT,MRI combined with PET-CT and other imaging examinations can guide the diagnosis,treatment and monitoring of recurrence and metastasis.