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
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
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
目的分析主动脉弓离断(IAA)合并中重度肺动脉高压患儿一期外科手术治疗效果。方法收集2010年1月至2017年9月在首都医科大学附属北京安贞医院小儿心脏中心行一期外科手术矫治的合并中重度肺动脉高压的IAA患儿33例病历资料。23例患儿合并...目的分析主动脉弓离断(IAA)合并中重度肺动脉高压患儿一期外科手术治疗效果。方法收集2010年1月至2017年9月在首都医科大学附属北京安贞医院小儿心脏中心行一期外科手术矫治的合并中重度肺动脉高压的IAA患儿33例病历资料。23例患儿合并重度肺动脉高压,口服肺动脉靶向药物治疗,下肢经皮血氧饱和度至少上升5%后手术。行端侧吻合21例,后壁端侧吻合+前壁心包片加宽12例。随访2~9年,分析治疗效果。结果术后死亡2例(6.1%),其中术后17 d(出院后第7天)、术后4个月各死亡1例。出院时、术后6个月及术后2年主动脉吻合口压差分别为(17±11)、(15±8)、(14±7)mm Hg(1 mm Hg=0.133 k Pa),不同时点间差异无统计学意义(P=0.152)。术后6个月及术后2年患儿平均肺动脉压力均明显低于术前[(38±7)、(24±5)mm Hg比(68±8)mm Hg],术后6个月患儿肺血管阻力明显低于术前[(493±248)dyn·s/cm^5比(1635±352)dyn·s/cm^5](均P<0.05)。结论一期外科手术治疗大龄IAA合并中重度肺动脉高压可以取得满意的手术疗效。围术期应用靶向药物降低肺动脉压力,选择恰当的手术方式是降低死亡率和防治术后吻合口狭窄的有效手段。展开更多
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.
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.
文摘目的分析主动脉弓离断(IAA)合并中重度肺动脉高压患儿一期外科手术治疗效果。方法收集2010年1月至2017年9月在首都医科大学附属北京安贞医院小儿心脏中心行一期外科手术矫治的合并中重度肺动脉高压的IAA患儿33例病历资料。23例患儿合并重度肺动脉高压,口服肺动脉靶向药物治疗,下肢经皮血氧饱和度至少上升5%后手术。行端侧吻合21例,后壁端侧吻合+前壁心包片加宽12例。随访2~9年,分析治疗效果。结果术后死亡2例(6.1%),其中术后17 d(出院后第7天)、术后4个月各死亡1例。出院时、术后6个月及术后2年主动脉吻合口压差分别为(17±11)、(15±8)、(14±7)mm Hg(1 mm Hg=0.133 k Pa),不同时点间差异无统计学意义(P=0.152)。术后6个月及术后2年患儿平均肺动脉压力均明显低于术前[(38±7)、(24±5)mm Hg比(68±8)mm Hg],术后6个月患儿肺血管阻力明显低于术前[(493±248)dyn·s/cm^5比(1635±352)dyn·s/cm^5](均P<0.05)。结论一期外科手术治疗大龄IAA合并中重度肺动脉高压可以取得满意的手术疗效。围术期应用靶向药物降低肺动脉压力,选择恰当的手术方式是降低死亡率和防治术后吻合口狭窄的有效手段。
