294例儿童眼眶占位病变病种分析

目的了解儿童眼眶占位病变的病种分类、疾病构成特征和分布情况。设计回顾性病例系列研究。研究对象1999年7月至2004年12月经我研究所确诊为眼眶占位病变的294例儿童患者病例资料。方法按照Shields分类方法，统计每类疾病中的病种和例数。统计良、恶性病变的比例。按年龄阶段分组，统计不同年龄阶段最常见的良、恶性病变。主要指标病理诊断或临床诊断结果。结果儿童眼眶占位病变可大致分为13类，以血管性、囊性和周围神经病变最常见。良性病变占88．1％，恶性肿瘤占11．9％。在0～4岁、5-9岁和10～14岁三个不同年龄阶段，毛细血管瘤、脉管瘤和静脉性血管瘤分别为最常见良性病变；横纹肌肉瘤在各年龄阶段均是最常见恶性肿瘤。结论儿童眼眶占位病变以良性为主，以血管性、囊性和周围神经病变多见。     

1492例眼眶占位性病变临床病理学分析

目的探讨北京同仁医院眼科中心眼眶占位性病变的疾病谱系和发病情况。设计回顾性病例系列。研究对象自1997年1月至2006年12月间北京同仁医院眼科中心病理室存档的1492例眼眶占位性病变。方法分析1492例眼眶占位性病变的临床病理资料,基本情况包括患者的年龄、性别及肿物的病理诊断。主要指标眼眶占位性病变的疾病谱系和发病情况。结果眼眶占位性病变依据组织来源主要分为15大类:囊肿性病变、脉管性病变、周围神经病变、视神经和脑膜病变、纤维组织病变、骨和软骨病变、脂肪组织病变、肌组织病变、泪腺病变、炎性病变、色素性病变、转移性肿瘤、淋巴增生性病变、继发性肿瘤、白血病和组织细胞系统病变、其它病变。其中良性病变1318例(88.3%),恶性病变174例(11.7%)。位于良性病变前5位的分别是皮样囊肿及表皮样囊肿、海绵状血管瘤、淋巴管瘤、泪腺混合瘤、慢性炎症和炎性假瘤;位于恶性肿瘤前5位的分别是淋巴瘤、泪腺囊腺癌、横纹肌肉瘤、黑色素瘤、皮脂腺腺癌。结论眼眶占位性病变涉及不同组织来源的病变。横纹肌肉瘤是儿童最常见的眶内恶性肿瘤,淋巴瘤是老年人最常见的眶内恶性肿瘤。(眼科,2007,16:398-402)     

儿童眼眶占位性病变的临床及病理组织学分析

目的：探讨儿童眼眶占位性病变的病理组织学分类及发病情况,为儿童眼眶占位性病变的诊断提供参考。
　　方法：回顾性分析天津医科大学第二医院1998-01/2012-12收治并经病理学证实的儿童眼眶占位性病变290例病例资料。
　　结果：选取的290例儿童眼眶占位性病变中以静脉性血管瘤最为常见,共58例(20.0%),其它常见病变依次为血管淋巴管瘤、皮样囊肿、神经纤维瘤、视神经胶质瘤、横纹肌肉瘤等。毛细血管瘤、静脉性血管瘤、视神经胶质瘤等有明显年龄倾向;视神经胶质瘤、静脉性血管瘤、横纹肌肉瘤等有明显性别倾向。
　　结论：儿童眼眶占位性病变以先天性和胚胎性为主,其中以静脉性血管瘤最为多见,某些病变具有明显的年龄及性别倾向。     

快速动态增强磁共振成像在眼眶占位性病变中的诊断价值

目的探讨快速动态增强磁共振成像(MRI)在眼眶占位性病变诊断中的价值。设计回顾性病例系列。研究对象21例眼眶占位性病例。方法患者眼眶占位性病变均行MRI快速动态增强扫描并得到病理证实。原始数据经GE Functool软件后处理,对时间信号强度曲线(TIC)、峰值时间、1分钟强化率及最大强化率等进行分析。主要指标时间信号强度曲线,1分钟强化率,最大强化率。结果眼眶良、恶性占位性病变的时间信号强度曲线有各自特点,16例良性病变中12例表现为持续升高型,5例恶性肿瘤中3例淋巴瘤均为平台型;恶性肿瘤的1分钟强化率(150.47±42.18)大于良性病变(1 01.37±43.02)(P=0.021);海绵状血管瘤有特殊的充填式强化模式。结论MRI快速动态增强检查有助于眼眶良、恶性占位性病变的诊断和鉴别诊断。(眼科,2007,16:305-308)     

233例眼眶占位性病变的组织病理学分析

目的 分析眼眶占位性病变的组织病理学分类.方法 对河南省眼科研究所2001年1月～2007年9月眼科病理室存档的233例眼眶占位性病变手术切除标本进行组织病理学分类.结果 良性病变191例(81.97%),其中前3位依次为海绵状血管瘤60例(25.75%),炎症22例(9.44%),皮样囊肿13例(5.58%).恶性病变42例(18.03%),其中原发性恶性肿瘤20例(8.97%),包括淋巴瘤10例(4.29%),泪腺上皮性恶性肿瘤4例(1.72%).横纹肌肉瘤3例(1.29%),纤维肉瘤1例(0.43%),其他2例(0.86%);继发性恶性肿瘤22例(9.87%),分别为眼睑皮脂腺癌眼眶蔓延9例(3.86%),眼睑鳞状细胞癌眼眶蔓延6例(2.58%),眼睑黑色素瘤眼眶蔓延3例(1.29%),视网膜母细胞瘤眶内蔓延3例(1.29%),眼睑基底细胞癌眼眶蔓延1例(0.43%).结论 眼眶占位性病变中脉管源性肿瘤最常见,其中最多见的是海绵状血管瘤,恶性病变中最多见的是淋巴瘤.     

141例泪腺占位性病变临床分析

目的：探讨141例泪腺占位性病变患者的病理类型和临床特点。方法：回顾性系列病例研究。选取2019年6月至2021年10月在吉林大学第二医院眼科中心诊治的141例泪腺区占位病变患者的临床资料，包括患者性别、年龄、临床表现、影像学特点及组织病理结果。依据病理诊断分类，探讨泪腺占位性病变的临床和影像学特点。组间比较采用独立样本t检验或非参数秩和检验；分类变量以例数、百分比表示，组间比较采用卡方检验和（或）Fisher确切概率法。结果：纳入患者中，男50例，女91例。按病变性质分类，良性病变117例（83.0%），最常见的是IgG4相关泪腺炎55例（39.0%）；恶性肿瘤24例（17.0%），最常见的是淋巴瘤10例。良性病变患者年龄（[ 45.8±15.7）岁]小于恶性肿瘤（[ 56.3±16.2） 岁]，差异有统计学意义（Z=-3.23, P=0.001）。按病变类型依次为炎性病变72例（51.1%），上皮性病变33例（23.4%），淋巴系统病变17例（12.1%）。比较泪腺上皮性病变、炎性病变、淋巴系统病变3组患者性别构成、临床表现（眼睑肿胀、眼球突出、运动障碍、视力下降）、影像学表现（双侧泪腺受累、眶骨质改变、鼻窦炎），差异均有统计学意义（均P<0.05）；3组在眼痛、眼外肌和三叉神经增粗，差异 无统计学意义。两两相比显示，泪腺上皮性病变较泪腺炎性病变的女性患者比例少，出现眼睑肿胀、泪腺双侧受累、鼻窦炎的患者比例少（χ2=17.01，P<0.001；χ2=32.34,P<0.001；χ2=10.43，P=0.001； χ2=10.12，P=0.001），而有眼球突出、运动障碍和视力下降、骨质破坏的患者比例较高（χ2=18.71，P<0.001；χ2=7.64，P=0.006；χ2=7.32，P=0.012）；泪腺上皮性病变患者与淋巴系统病变相比，前者出现眼睑肿胀、双侧泪腺受累的患者比例低，眼球突出的患者比例较高，差异有统计学意义（χ2=15.93，P<0.001；χ2=6.38，P=0.012；χ2=10.14，P=0.001）；泪腺炎性病变与淋巴系统病变性仅在泪腺双侧受累比例差异有统计学意义(χ2=10.43，P=0.001）；淋巴系统病变与炎性及上皮性病变相比，其余各组间临床和影像学的表现差异无统计学意义。儿童泪腺占位性病变7例中未见上皮性病变。结论：泪腺占位性病变中最常见的是炎性病变，其次是上皮性病变；良性病变平均年龄小于恶性肿瘤；儿童泪腺病变类型分布与成人不同；泪腺炎性病变与上皮性病变的临床和影像学表现差异较大，相对容易鉴别。     

眼眶占位性病变36例手术分析

目的探讨眼眶占位性病变的临床诊断，手术治疗及其组织病理学分类。方法对2004年1月～2006年5月手术治疗的36例（36眼）眼眶占位性病变的性别、年龄、眼别及病理检查结果进行分析。结果恶性肿瘤3例，良性病变33例。组织病理学检查：恶性肿瘤3例均为非何杰金氏淋巴瘤；良性者中海绵状血管瘤9例，泪腺多形性腺瘤8例，静脉性血管瘤3例，炎性假瘤3例，囊肿3例，皮脂腺囊肿2例，淋巴管瘤、视神经胶质瘤、神经纤维瘤、骨瘤及颗粒细胞瘤各1例。在泪腺多形性腺瘤中浸润性生长3例，恶变1例。结论眼眶占位性病变种类繁多，良性肿瘤中以海绵状血管瘤多见，可采用前路开眶或外侧壁开眶顺利摘除肿瘤，次为泪腺多形性腺瘤，并且容易因手术切除不彻底或术中肿瘤破碎残留肿瘤细胞而改变生长方式，甚至于恶变。炎性假瘤手术完整切除较难，多要进一步作术后治疗。神经源性肿瘤早期就对视力损伤较大。     

眼眶囊性病变134例临床诊治分析

目的 描述眼眶囊性病变病例的临床表现、诊断和治疗.方法 回顾性研究2001年1月至2009年3月期间诊治的眼眶囊性病变134例,分析其临床特征,影像学表现和手术治疗效果等.结果 134例眼眶囊性病变中,男性68例;女性66例,右眶63例;左眶71例;年龄6个月至84岁,平均42岁.最常见症状为肿块、眼球突出和复视等.超声波检查示肿物多呈边界清楚的无回声或低回声区,内部无明显血流信号.CT示囊肿内密度较均匀,CT值-198Hu～77.5Hu,增强扫描示囊壁增强,而囊内容物不被强化.MRI检查T1WI为低或等信号,T2WI呈高信号.全部病例经手术完整切除或部分切除等治疗.术后病理诊断眼眶皮样囊肿46例,表皮样囊肿58例,黏液囊肿11例,单纯性眼眶血肿12例,眼眶脓肿3例,寄生虫性囊肿2例,泪腺起源的单纯性上皮细胞囊肿及先天性囊性眼各1例.术后随访3月至8年,平均2.10年,治愈率98.50%,复发率1.50%.结论 眼眶囊性病变的诊断应综合病史和临床表现,影像学检查有助于准确的诊断,手术治疗效果好.     

2000至2021年眼眶占位性病变1 913例的临床病理学构成分析

目的探讨眼眶占位性病变的临床病理学构成情况。方法本研究为回顾性病例系列研究。收集浙江大学医学院附属第二医院2000年1月至2021年12月因眼眶占位性病变行切除手术的1 913例患者的1 913份组织标本资料进行病理学分析, 按照不同组织发生学类型、病变性质及年龄段分类, 分析其构成情况。结果 1 913例患者中, 男性913例(47.7%), 女性1 000例(52.3%);良性病变1 489例(77.8%), 恶性病变424例(22.2%)。按组织发生学类型分类:脉管性病变占首位, 为521例(27.2%), 囊肿性病变407例(21.3%), 淋巴增生性病变277例(14.5%), 泪腺来源182例(9.5%), 炎性反应病变121例(6.3%)。按病变性质分类:1 489例良性病变中, 海绵状血管瘤占首位(275例, 占全部患者14.4%), 其后依次为皮样囊肿225例(11.8%), 其他类血管瘤199例(10.4%), 表皮样囊肿136例(7.1%), 良性泪腺混合瘤134例(7.0%)。恶性病变中, 淋巴瘤210例(占全部患者11.0%), 皮脂腺癌47例(2.5%)。全...     

泪囊区占位病变13例临床分析

目的 探讨泪囊区占位病变的临床诊断及治疗方法。方法 对10年间诊治的13例泪囊区占位病变的临床表现、影像学特征和治疗进行分析。结果 13例病变中，根据病变特点分囊肿性病变7例、实体性病变6例。临床上以溢泪、泪囊区肿物、血性分泌物、疼痛等为主要特征，B超及CT检查提示病变的形态、性质。结论 泪囊区占位病变根据临床表现及影像学检查，可早期明确诊断。治疗以手术切除为主，酌情辅以放、化疗，预后良好。     

Klinische und neuroradiologische Diagnostik bei Orbitatumoren

Exophthalmus is the leading sign of space-occupying lesions of the orbit. Patients may further present with lid swelling, impaired ocular motility and optic neuropathy including a relative afferent pupillary defect, compressive optic disc edema or optic atrophy. Orbital tumors can be classified into various categories depending on the etiology, as lymphoproliferative lesions (in particular non-Hodgkin's lymphoma as the most common malignant orbital tumor of adulthood), optic nerve and meningeal lesions, lacrimal gland lesions, secondary orbital tumors which extend to the orbit from neighboring structures and metastases. Slightly less common are vasculogenic and cystic lesions including cavernous hemangioma as the most common benign orbital tumor of adulthood and dermoid cysts as the most common benign orbital tumor of childhood. Rhabdomyosarcoma is the most common malignant orbital tumor of childhood but has a low total incidence. Orbital tumors might not only cause symptoms like pain, diplopia and loss of visual acuity but may also lead to esthetically disfiguring changes. Particular attention should be paid to underlying systemic diseases and generalized tumor diseases. This article illustrates the approach to a detailed clinical and neuroradiological assessment which is mandatory for the care of orbital tumor patients.     

Space-occupying orbital masses in children. A review of 250 consecutive biopsies

A review was done of 250 consecutive biopsies for orbital space-occupying lesions in children. Benign cystic lesions were by far the most common, accounting for 52% of the lesions. Inflammatory lesions, usually biopsied to exclude the diagnosis of rhabdomyosarcoma, accounted for 16%. Among the 250 biopsies there were 14 primary malignant tumors (6%), ten of which were rhabdomyosarcoma. Although rhabdomyosarcoma is not the most common space-occupying orbital lesion in children, clinicians should be familiar with its clinical features and proceed with immediate biopsy followed by appropriate irradiation and chemotherapy if the diagnosis is established. However, biopsy should only be undertaken if there is reasonable suspicion that the lesion could be rhabdomyosarcoma or other childhood orbital malignancy.     

Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1

OBJECTIVE: To determine the incidence of specific orbital tumors based on patients referred to an ocular oncology center. DESIGN: Retrospective, observational case series. PARTICIPANTS: A total of 1264 consecutive patients referred to an ocular oncology service because of space-occupying orbital lesions. METHODS: A retrospective chart review was carried out for 1264 consecutive patients referred for a suspected orbital mass over a 30-year period. The lesions were grouped into general categories, as shown in "Results." The specific diagnosis in each case was based on clinical findings, computed tomography scan results, magnetic resonance imaging results, and histopathologic analysis results, when available. The number and percentage of benign and malignant tumors per age group also was determined. MAIN OUTCOME MEASURES: The incidence of orbital tumors and pseudotumors. RESULTS: Among 1264 consecutive patients, the number and percentage of lesions in each general category were as follows: cystic, 70 cases (6%); vasculogenic, 213 cases (17%); peripheral nerve lesions, 23 (2%); optic nerve and meningeal tumors, 105 (8%); fibrocytic lesions, 13 (1%); osseous and fibro-osseous tumors, 21 (2%); cartilaginous lesions, 1 (<1%); lipocytic and myxoid lesions, 64 (5%); myogenic tumors, 36 (3%); lacrimal gland lesions, 114 (9%); primary melanocytic lesions, 11 (<1%); metastatic tumors, 91 (7%); lymphoma and leukemia lesions, 130 (10%); secondary orbital tumors, 142 (11%); histiocytic lesions, 17 (1%); thyroid-related orbitopathy, 67 cases (5%); other inflammatory lesions, 133 cases (11%); and miscellaneous other lesions, 13 (1%). The most common diagnoses were: lymphoid tumor (139 cases;11%), idiopathic orbital inflammation (135 cases; 11%), cavernous hemangioma (77 cases; 6%), lymphangioma (54 cases; 4%), meningioma (53 cases; 4%), optic nerve glioma (48 cases; 4%), metastatic breast cancer (44 cases;4%), orbital extension of uveal melanoma (41 cases; 3%), capillary hemangioma (36 cases;3%), rhabdomyosarcoma (35 cases; 3%), dermolipoma (31 cases; 3%), herniated orbital fat (30 cases; 2%), dermoid cyst (26 cases; 2%), varix (26 cases; 2%), dacryops (19 cases; 2%), and other less common lesions. Of the 1264 lesions, 810 (64%) were benign and 454 (36%) were malignant. The percentage of malignant lesions was 20% in children (age range, 0-18 years), 27% in young adults and middle-aged patients (age range, 19-59 years), and 58% in older patents (age range, 60-92 years). Rhabdomyosarcoma was the most common malignancy in children, representing 3% of all orbital masses, and lymphoma was the most common malignancy in older patients, representing 10% of cases. CONCLUSIONS: A variety of tumors and pseudotumors can involve the orbit. In this series of 1264 lesions, 64% were benign and 36% were malignant. The percentage of malignant tumors increased with age, with malignancies being common in older patients because of the higher incidence of lymphoma and metastasis in the elderly.     

Orbitale Raumforderungen im Wachstumsalter

Background

The aim of this retrospective study was to determine the incidence of and surgical strategies used for treating orbital tumours in children and adolescents.

Patients and Methods

A retrospective chart review was carried out for 22 patients between the ages of 2 weeks and 18 years who were treated in the years 1997–2007 for suspected space-occupying orbital lesions. The specific investigation methods, therapy, and treatment results were described.

Results

The most frequent orbital lesions in the children in this study were mesenchymal tumours; the second most common were dermoids. Nineteen patients were surgically treated. Three children with malignant tumours underwent radiochemotherapy after surgery, and two died in the first year after diagnosis. Two other children with benign tumours suffered recurrence of their disease.

Conclusion

The variety of orbital diseases in paediatric patients includes benign and malignant tumours as well as nonneoplastic lesions. The treatment of orbital disease is a diagnostic and therapeutic challenge for the attending physician.     

Clinicopathological analysis of 719 pediatric and adolescents’ ocular tumors and tumor-like lesions: a retrospective study from 2000 to 2018 in China

AIM: To analyze the retinal proteomes with and without conbercept treatments in mice with oxygen-induced retinopathy (OIR) and identify proteins involved in the molecular mechanisms mediated by conbercept. METHODS: OIR was induced in fifty-six C57BL/6J mouse pups and randomly divided into four groups. Group 1: Normal17 (n=7), mice without OIR and treated with normal air. Group 2: OIR12/EXP1 (n=14), mice received 75% oxygen from postnatal day (P) 7 to 12. Group 3: OIR17/Control (n=14), mice received 75% oxygen from P7 to P12 and then normal air to P17. Group 4: Lang17/EXP2 (n=21), mice received 75% oxygen from P7 to P12 with intravitreal injection of 1 μL conbercept at the concentration of 10 mg/mL at P12, and then normal air from P12 to P17. Liquid Chromatography-Mass Spectrometry (LC-MS)/MS data were reviewed to find proteins that were up-regulated after the conbercept treatment. Gene ontology (GO) analysis was performed of conbercept-mediated changes in proteins involved in single-organism processes, biological regulation, cellular processes, immune responses, metabolic processes, locomotion and multiple-organism processes. RESULTS: Conbercept induced a reversal of hypoxia-inducible factor 1 signaling pathway as revealed by the Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis and also induced down-regulation of proteins involved in blood coagulation and fibrin clot formation as demonstrated by the Database for Annotation, Visualization and Integrated Discovery (DAVID) and the stimulation of interferon genes studies. These appear to be risk factors of retinal fibrosis. Additional conbercept-specific fibrosis risk factors were also identified and may serve as therapeutic targets for fibrosis. CONCLUSION: Our studies reveal that many novel proteins are differentially regulated by conbercept. The new insights may warrant a valuable resource for conbercept treatment.     

Orbital tumors in the older adult population

PURPOSE: To determine the types and frequency of orbital tumors in the older adult population. DESIGN: Retrospective consecutive case series. PARTICIPANTS: Two hundred consecutive patients aged 60 years or older with an orbital tumor managed at a tertiary referral center. MAIN OUTCOME MEASURES: The relative incidence and management of orbital tumors in patients aged 60 years or older. RESULTS: Of approximately 950 consecutive patients with orbital space-occupying lesions managed over a 25-year period on the Oncology Service, 200 patients (21%) were 60 years or older at the time of diagnosis. The orbital tumor was unilateral in 183 patients (92%) and bilateral in 17 patients (8%). The most common clinical features at presentation included mass in 51 patients (26%), proptosis in 36 (18%), and pain in 30 (15%). The mean duration of symptoms was 11 months before referral. The orbital tumor was malignant in 125 patients (63%) and benign in 75 (27%). The most common diagnoses were malignant lymphoma in 47 patients (24%), idiopathic orbital inflammation in 19 (10%), and cavernous hemangioma in 15 (8%). The diagnosis was made with clinical findings and imaging studies in 58 patients (29%) and additional histopathologic examination in 142 patients (71%). Of 217 tumors, the main tumor location was in the superior half of the orbit in 106 lesions (49%), in the anterior orbit in 100 (46%), and in the extraconal space in 135 (62%). Imaging studies showed a mass with well-defined margins in 90 tumors (41%) and ill-defined margins in 127 (59%). The tumors were solid in 203 cases (94%), cystic in 6 (3%), and mixed in 8 (3%). Over a mean of 31 months follow-up, 50 (25%) patients had orbital tumor-related systemic involvement (metastasis or lymphoma), and 150 (75%) remained free of systemic involvement. CONCLUSIONS: Orbital tumors in the senior adult population are malignant in 63% of cases. Malignant lymphoma is the most common tumor in this age group, accounting for 24% of cases. Overall, 25% of patients have systemic problems related to the orbital process develop, so systemic evaluation is warranted.