AIM:To analyze and summarize the clinical and pathological features of ciliary body medulloepithelioma.METHODS:The clinical and pathological data of 11 patients(11 eyes) who were diagnosed with ciliary body medulloepi...AIM:To analyze and summarize the clinical and pathological features of ciliary body medulloepithelioma.METHODS:The clinical and pathological data of 11 patients(11 eyes) who were diagnosed with ciliary body medulloepithelioma at Beijing Tongren Hospital,Capital Medical University,from 2007 to 2021 were retrospectively analyzed.RESULTS:The initial symptoms of 11 patients included vision loss(6 eyes),atrophia bulbi(1 eye),proptosis(2 eyes),and leukocoria(2 eyes).Most patients suffered with corneal opacity,anterior chamber flare and hyphema.Iris neovascularization and synechia,complicated cataract,and secondar y glaucoma occurred in several cases.Three patients even had lens subluxation and retinal detachment.B-scan ultrasonography showed vitreous opacity and a medium-high uneven echo mass in the eyeball.Ultrasound biomicroscopy examination showed a spherical or hemispherical ciliary body mass with uneven internal echoes and irregular cystic spaces.All of the 11 patients were diagnosed with malignant ciliary body medulloepithelioma by pathological evidence.In this study,6 patients had enucleation(2 patients had systemic chemotherapy after surgery),and the other 5 patients had local tumor resection(1 patient had plaque radiotherapy after surgery).CONCLUSION:Ciliary body medulloepithelioma is a rare intraocular tumor and may be easily confused with retinoblastoma.Analyzing the clinical and pathological features of ciliary body medulloepithelioma is useful to further understand ciliary body medulloepithelioma,and can make an accurate diagnosis and better treatment.展开更多
AIM:To explore the clinical and pathological characteristics of thirteen patients with orbital liposarcoma.METHODS:The clinical history data of thirteen patients diagnosed as orbital liposarcoma at Beijing Tongren Hos...AIM:To explore the clinical and pathological characteristics of thirteen patients with orbital liposarcoma.METHODS:The clinical history data of thirteen patients diagnosed as orbital liposarcoma at Beijing Tongren Hospital,from 2006 to 2021 were collected and analyzed.The data includes age,gender,affected orbital side,course of disease,status of disease(primary or recurrent),clinical manifestations,preoperative visual acuity,operative treatment,the relations between liposarcoma and surrounding tissue,longest diameter of liposarcoma,histological subtype,immunohistochemical indicators,follow-up treatment and prognosis.RESULTS:The initial symptoms are diverse.Proptosis is the most frequent chief complaint and the others included vision loss,epiphora,diplopia,and eyelid palpable mass.Results of imaging examination[computed tomography(CT)or magnetic resonance imaging(MRI)]showed orbital mass.In terms of treatment,10 patients received tumor resection,and the mean longest diameter of the tumor was 3.39±1.36 cm.The other 3 patients had optic nerve invaded,so they received orbital exenteration.Pathological examination results confirmed the diagnose of liposarcoma for 13 patients.Six patients displayed as myxoid type,and three patients in each type of dedifferentiated and welldifferentiated type.One patient was verified as pleomorphic,which was a rare type of liposarcoma.All of the patients showed Vimentin positive,and most showed CD34 and S-100 positive.Besides,four patients showed smooth muscle actin positive.All thirteen patients were alive.CONCLUSION:Orbital liposarcoma is a rare disease and it has no specific clinical manifestation.The diagnosis of liposarcoma should be considered when proptosis and orbital mass occurred in orbit.It is recommended to perform pathological examination to achieve early detection and early treatment.展开更多
Dear Editor,We present an exceptionally rare case about bilateral medulloepithelioma of the ciliary body.This case was approved by Ethics Committee of Capital Medical University.Written informed consent was obtained f...Dear Editor,We present an exceptionally rare case about bilateral medulloepithelioma of the ciliary body.This case was approved by Ethics Committee of Capital Medical University.Written informed consent was obtained from the patients.Discovering leukocoria in both eyes and proptosis in right eye for 2mo by parents in a 4.5-month-old boy.He had undergone no treatment elsewhere except magnetic resonance imaging examination before coming to Beijing Tongren Hospital.展开更多
目的:总结先天性心脏病术后主动脉瓣下狭窄的手术经验及手术疗效。方法:回顾性分析2011年2月至2017年12月,行先心病术后主动脉瓣下狭窄外科矫治的22例患儿临床资料,其中男性13例,女性9例。原发心脏畸形包括室间隔缺损、心内膜垫缺损、...目的:总结先天性心脏病术后主动脉瓣下狭窄的手术经验及手术疗效。方法:回顾性分析2011年2月至2017年12月,行先心病术后主动脉瓣下狭窄外科矫治的22例患儿临床资料,其中男性13例,女性9例。原发心脏畸形包括室间隔缺损、心内膜垫缺损、主动脉弓缩窄、右心室双出口、法洛四联症、动脉导管未闭等。初次手术矫治年龄2个月~4岁(中位年龄0.85岁),术后主动脉瓣下狭窄矫治年龄1.6~15.9岁,平均(7.49±4.09)岁,与初次手术间隔1~15.3年,平均(6.22±3.63)年。结果:所有患儿均行主动脉瓣下狭窄矫治术,无术中死亡;跨左心室流出道压差从术前52~169 mm Hg (1 mmHg=0.133 kPa),平均(94.09±25.02)mm Hg降至术后5~47 mm Hg,平均(16.19±10.87)mm Hg(P<0.05)。1例术后Ⅲ°房室传导阻滞并安装永久性心脏起搏器。所有患儿均常规随访1~8年,术后均无症状,心功能正常,无远期死亡。结论:先天性心脏病术后的主动脉瓣下狭窄考虑左心室流出道血流动力学紊乱造成,其原发畸形复杂多变,再次手术时间间隔较长,且术后仍有复发可能。展开更多
文摘AIM:To analyze and summarize the clinical and pathological features of ciliary body medulloepithelioma.METHODS:The clinical and pathological data of 11 patients(11 eyes) who were diagnosed with ciliary body medulloepithelioma at Beijing Tongren Hospital,Capital Medical University,from 2007 to 2021 were retrospectively analyzed.RESULTS:The initial symptoms of 11 patients included vision loss(6 eyes),atrophia bulbi(1 eye),proptosis(2 eyes),and leukocoria(2 eyes).Most patients suffered with corneal opacity,anterior chamber flare and hyphema.Iris neovascularization and synechia,complicated cataract,and secondar y glaucoma occurred in several cases.Three patients even had lens subluxation and retinal detachment.B-scan ultrasonography showed vitreous opacity and a medium-high uneven echo mass in the eyeball.Ultrasound biomicroscopy examination showed a spherical or hemispherical ciliary body mass with uneven internal echoes and irregular cystic spaces.All of the 11 patients were diagnosed with malignant ciliary body medulloepithelioma by pathological evidence.In this study,6 patients had enucleation(2 patients had systemic chemotherapy after surgery),and the other 5 patients had local tumor resection(1 patient had plaque radiotherapy after surgery).CONCLUSION:Ciliary body medulloepithelioma is a rare intraocular tumor and may be easily confused with retinoblastoma.Analyzing the clinical and pathological features of ciliary body medulloepithelioma is useful to further understand ciliary body medulloepithelioma,and can make an accurate diagnosis and better treatment.
基金Supported by National Natural Science Foundation of China(No.82000862,No.82201214)Shaanxi Key Research and Development Program(No.2021SF-156).
文摘AIM:To explore the clinical and pathological characteristics of thirteen patients with orbital liposarcoma.METHODS:The clinical history data of thirteen patients diagnosed as orbital liposarcoma at Beijing Tongren Hospital,from 2006 to 2021 were collected and analyzed.The data includes age,gender,affected orbital side,course of disease,status of disease(primary or recurrent),clinical manifestations,preoperative visual acuity,operative treatment,the relations between liposarcoma and surrounding tissue,longest diameter of liposarcoma,histological subtype,immunohistochemical indicators,follow-up treatment and prognosis.RESULTS:The initial symptoms are diverse.Proptosis is the most frequent chief complaint and the others included vision loss,epiphora,diplopia,and eyelid palpable mass.Results of imaging examination[computed tomography(CT)or magnetic resonance imaging(MRI)]showed orbital mass.In terms of treatment,10 patients received tumor resection,and the mean longest diameter of the tumor was 3.39±1.36 cm.The other 3 patients had optic nerve invaded,so they received orbital exenteration.Pathological examination results confirmed the diagnose of liposarcoma for 13 patients.Six patients displayed as myxoid type,and three patients in each type of dedifferentiated and welldifferentiated type.One patient was verified as pleomorphic,which was a rare type of liposarcoma.All of the patients showed Vimentin positive,and most showed CD34 and S-100 positive.Besides,four patients showed smooth muscle actin positive.All thirteen patients were alive.CONCLUSION:Orbital liposarcoma is a rare disease and it has no specific clinical manifestation.The diagnosis of liposarcoma should be considered when proptosis and orbital mass occurred in orbit.It is recommended to perform pathological examination to achieve early detection and early treatment.
文摘Dear Editor,We present an exceptionally rare case about bilateral medulloepithelioma of the ciliary body.This case was approved by Ethics Committee of Capital Medical University.Written informed consent was obtained from the patients.Discovering leukocoria in both eyes and proptosis in right eye for 2mo by parents in a 4.5-month-old boy.He had undergone no treatment elsewhere except magnetic resonance imaging examination before coming to Beijing Tongren Hospital.
文摘目的:总结先天性心脏病术后主动脉瓣下狭窄的手术经验及手术疗效。方法:回顾性分析2011年2月至2017年12月,行先心病术后主动脉瓣下狭窄外科矫治的22例患儿临床资料,其中男性13例,女性9例。原发心脏畸形包括室间隔缺损、心内膜垫缺损、主动脉弓缩窄、右心室双出口、法洛四联症、动脉导管未闭等。初次手术矫治年龄2个月~4岁(中位年龄0.85岁),术后主动脉瓣下狭窄矫治年龄1.6~15.9岁,平均(7.49±4.09)岁,与初次手术间隔1~15.3年,平均(6.22±3.63)年。结果:所有患儿均行主动脉瓣下狭窄矫治术,无术中死亡;跨左心室流出道压差从术前52~169 mm Hg (1 mmHg=0.133 kPa),平均(94.09±25.02)mm Hg降至术后5~47 mm Hg,平均(16.19±10.87)mm Hg(P<0.05)。1例术后Ⅲ°房室传导阻滞并安装永久性心脏起搏器。所有患儿均常规随访1~8年,术后均无症状,心功能正常,无远期死亡。结论:先天性心脏病术后的主动脉瓣下狭窄考虑左心室流出道血流动力学紊乱造成,其原发畸形复杂多变,再次手术时间间隔较长,且术后仍有复发可能。
