眼眶乳头状血管内皮增生的临床病理学观察

Clinicopathological features of orbital intravascular papillary endothelial hyperplasia

目的分析眼眶乳头状血管内皮增生的临床病理学特点。设计回顾性病例系列。研究对象2006-2019年天津市眼科医院经病理检查证实的眼眶乳头状血管内皮增生病例9例。方法回顾患者的临床和病理学资料。主要指标临床资料包括患者年龄、性别、病史、临床表现、B超、彩色多普勒超声、CT和MRI检查结果。病理学资料包括肿瘤大体形态、肿瘤体积、病理形态和免疫组织化学特点。结果9例患者中,女性7例;平均发病年龄(46.8±11.0)岁。多数病例病史不超过2个月。眼球突出9/9例,眼球运动受限3/9例。B超和彩色多普勒超声检查显示眼球后类圆形占位性病变,回声均匀一致,彩色多普勒超声检查显示肿瘤内部无明显血流信号。CT检查显示8例肿物位于眼眶后部肌锥内,1例位于肌锥外;肿物呈类圆形,密度均匀,边界清楚。MRI检查3例,显示眶肌锥内肿物呈等T1较长T2信号。肿物外观呈灰紫色或灰黄色,包膜完整,与周围组织无明显粘连,最大直径10~20 mm。病理特点为附着于血管壁有很多纤细的乳头向腔内生长,乳头轴心为纤维素性或胶原化的纤维组织,乳头表面衬覆有单层、稍肥胖的内皮细胞,其对CD31和CD34呈阳性表达。随诊8/9例无复发,1例术后7年复发。结论眼眶乳头状血管内皮增生是一种非常少见的血管瘤性病变,多发生于成年人眼眶后部肌锥内,肿物为单发、边界清楚,局部切除后很少复发。(眼科,2021,30:51-55)

Objective To describe the clinicopathological features of orbital intravascular papillary endothelial hyperplasia(IPEH).Design Retrospective case series.Participants Nine cases of orbital IPEH retrospectively reviewed in Tianjin Eye Hospital in 2006–2019.Methods The clinical and pathological features were reviewed.The clinical data were evaluated for patient age,gender,clinical history,clinical features,Grayscale and color Doppler ultrasound,CT and MRI.Main Outcome Measures Pathological data,including gross presentation,tumor size,histopathological and immunohistochemical features were also reviewed.Results The study group included 7 females and 2 males.The average age at diagnosis was 46.8±11.0 years.The duration of symptoms before seeing an ophthalmologist was less than 2 months in most cases(7/9),and the most common symptoms were proptosis(9/9)and decreased eye motility(3/9).Grayscale and color Doppler US examinations showed an oval or round lesion with homogeneous echogenicity.No detectable vessels were found inside tumors by color Doppler US examinations.CT scanning(all 9 cases)revealed a well-circumscribed mass with homogeneous soft tissue density.Most lesions(8/9)located in retrobulbar muscle cone,however one case was extraconal.MRI(3 cases)showed isointense T1 signal and hyperintense T2 signal.The appearance of the tumor was gray-purple or gray-yellow,the capsule was intact,and there was no obvious adhesion to the surrounding tissues.The diameter of tumors was 10~20 mm.Photomicrograph showed the multiple small papillary projections lined by a single layer of plump endothelial cells and supported by fibrous connective tissue core,and multiple vascular channels.The endothelial cells were positive for CD31 and CD34.At the follow-up,8 cases had no recurrence,and only 1 case recurred after 7 years of surgical excision.Conclusion Orbital IPEH was a very rare vascular lesion,which often occurred in the muscle cone of the adult orbit.The tumor was single,the boundary is clear,and there was little recurrence after surgical excision.(Ophthalmol CHN,2021,30:51-55)