目的：探讨眼部转移性透明细胞肾细胞癌(clear-cell renal cell carcinoma，CC-RCC)的临床病理特点。方法：选取复旦大学附属眼耳鼻喉科医院2010年1月至2020年12月收治的5例并经病理学检查证实的眼部转移性CC-RCC患者的临床病理资料，包括病史、临床表现、影像学检查、病理形态学特点、免疫表型及随访结果等，并进行回顾分析。结果：5例患者中3例为脉络膜转移性CC-RCC，均为男性，年龄51~62岁，均表现为右眼前黑影伴视力下降，病程为1~6个月，术前检查视力均为眼前手动，眼底见视网膜下隆起肿块伴视网膜脱离。B超显示球内隆起肿物，中等回声，考虑脉络膜黑色素瘤。其中例2在2年前有左侧肾CC-RCC切除病史，术后1年转移至肺。3例患者均行眼球摘除加义眼座植入术。病理学形态及免疫组织化学染色结果提示为球内恶性肿瘤，考虑转移性CC-RCC，建议在肾等处寻找原发灶。术后例1腹部CT检查发现左肾占位，考虑肾癌。胸部CT检查示两下肺多个转移瘤。例3术后PET-CT发现左肾占位，手术切除后证实为左肾CC-RCC。2例为眼眶转移性CC-RCC，例4为女性，56岁，右眼红肿伴眼球突出2个月，2个月前行右肾CC-RCC切除术，PEC-CT提示右侧眼眶转移伴骨质破坏。例5为男性，65岁，左眼眉弓处肿物3年，7年前行左肾癌摘除术，后肺部转移。所有5例患者手术切除标本病理学检查均示肿瘤细胞细胞质透明或颗粒状，呈实性片状和腺样分布，间质血管丰富，免疫组织化学表达CK、VIM、CD10和PAX-8等标记提示CC-RCC转移。结论：CC-RCC可以转移至脉络膜或眼眶，病理学上需要和其他眼部具有透明细胞特征的原发和转移性肿瘤相鉴别。

Objective: To evaluate the clinicopathological features of ocular metastatic clear-cell renal cell carcinoma.Methods: Data of 5 patients (5 eyes) with ocular metastatic clear-cell renal cell carcinoma treated and diagnosed at Eye & ENT Hospital of Fudan University from January 2010 to December 2020 were retrospectively analyzed for medical history, clinical features, imaging examinations, pathomorphological features, immunophenotypes and survival outcomes. Results: There were 3 males of choroidal metastatic clear-cell renal cell carcinoma with age from 51 to 62 years old. They all presented with shadow before the eye and reduced visual acuity of the right eye for 1 to 6 months. On examination the visual acuity was hand movement in front of the affected eye. Fundus examination showed a subretinal elevated mass with retinal detachment. B-scan ultrasound demonstrated an intraocular mass with medium internal reflectivity suspected of choroidal melanoma. Case 2 reported a history of clear-cell renal cell carcinoma treated with a left nephrectomy 2 years ago and developed lung metastasis 1 year ago. Three patients all underwent enucleation and prosthesis implantation. Histopathological and immunohistochemical examinations showed intraocular malignant tumor suggestive of clear-cell renal cell carcinoma which needed further examinations to confirm the primary tumor. Postoperative computed tomography scan of the abdomen for case 1 revealed a mass of the left kidney highly suggestive of a renal cell carcinoma. The computed tomography scan of the chest revealed multiple lesions suggestive of lung metastasis. Postoperative PET-CT scan of case 3 revealed a mass of the left kidney which was confirmed to be clear-cell renal cell carcinoma histopathologically. There were 2 patients of orbital metastatic clear-cell renal cell carcinoma. One 56-year-old female patient (Case 4) presented with swelling, redness and proptosis of the right eye for 2 months. Two months ago, her right kidney was resected for the diagnosis of clear-cell renal cell carcinoma. PEC-CT revealed metastasis to the right orbit with bone destruction. Another 65-year-old male patient (Case 5) presented with palpable mass of the left eyebrow for 3 years. He had left nephrectomy for renal cell carcinoma 7 years earlier and metastasis to the lung later. Histopathology of all 5 cases demonstrated uniform cells with clear or granular cytoplasm in solid and glandular arrangement surrounded by a rich vascular network. Immunohistochemical positivity for the biomarkers CK, Vimentin, CD10 and PAX-8 confirmed the diagnosis of metastatic clear-cell renal cell carcinoma.Conclusion: Clear-cell renal cell carcinoma can metastasize to the choroid or orbit. It should be differentiated from the other ocular primary and metastatic tumors with clear-cell appearance histopathologically.

目的：分析46例双眼视网膜母细胞瘤(retinoblastoma，RB)的临床特点及治疗效果。方法：回顾性分析2008年12月至2019年12月重庆市陆军军医大学陆军特色医学中心收治的46例接受静脉化疗联合经瞳孔温热疗法(transpupillary thermotherapy，TTT)或眼摘治疗的双眼RB住院患儿的临床资料，对患儿的保眼率、摘眼率、视力情况及化疗不良反应进行评估。结果：46例患儿中，男27例，女19例，初诊年龄为(13.21±11.13)个月。单纯化疗10例，化疗+TTT治疗11例，化疗+TTT+眼摘治疗17例，化疗+冷凝治疗2例，化疗+冷凝+眼摘治疗6例。46例92眼总保眼率73.1%(57/78)，残留视力眼占64.1%(50/78)，各期保眼率：A、B期均100.0%，C期86.7%，D期94.1%，E期35.7%。手术摘除24眼，总摘眼率26.1%(24/92)，E期手术摘除21眼，占E期患眼60.0%(21/35)。平均化疗(4.1±1.9)次，化疗的骨髓抑制主要表现为白细胞减少、血小板减少及血红蛋白减少。46例患儿随访时间(35.4±23.8)个月，死亡7例，总病死率15.2%(7/46)；存活39例，总存活率为84.8%(39/46)，5年累积生存率为80.2%。结论：静脉化疗联合局部治疗总体疗效较好，在双眼RB患儿治疗中占据重要地位。化疗具有骨髓抑制作用，停止化疗后骨髓抑制逐渐恢复。

Objective: To analyze the clinical characteristics and therapeutic effect of 46 patients with bilateral retinoblastoma(RB). Methods: The clinical data of 46 patients with bilateral retinoblastoma who received intravenous chemotherapy combined with transpupillary thermotherapy (TTT) or enucleation from December 2008 to December 2019 in our department were analyzed retrospectively. The eye salvage rate, enucleation rate, visual acuity, and chemotherapy side effects were evaluated. Results: The 46 enrolled patients were 27 males and 19 females, at an average age of (13.21±11.13) months at the first visit. Among them, 10 received chemotherapy, 11 received chemotherapy combined with TTT, 17 received chemotherapy combined with TTT and enucleation,and 2 received chemotherapy combined with freezing, 6 received chemotherapy combined with freezing and enucleation. After treatment, 58 eyes were salvaged, with a total salvage rate of 73.1% (57/78), and the eyes that preserved vision account for 64.1% (50/78). The eye salvage rate in each stage were 100.0% for stage A and B, 86.7% for stage C, and 94.1% for stage D, 35.7% for stage E. Twenty-four eyes were enucleated, with a total enucleation rate of 26.1% (24/92), and among 35 eyes at stage E, 21 eyes were enucleated, accounting for 60.0%(21/35). The average time of chemotherapy was 4.1±1.9 and the myelosuppressive effects of chemotherapy include leucopenia, thrombocytopenia and hemoglobinopenia. During the mean follow-up time of (35.4±23.8) months, 7 (15.2%) patients died, and 39 (84.8%) cases survived. The 5-year cumulative survival rate was 80.2%. Conclusion: Intravenous chemotherapy combined with local treatment has a good overall effect and plays an important role in the treatment of bilateral retinoblastoma. Intravenous chemotherapy leads to myelosuppression, and the myelosuppression gradually recovered after stopping intravenous chemotherapy.

目的：探讨眼眶黏膜相关淋巴组织(mucosa-associated lymphoid tissue，MALT)结外边缘区B细胞淋巴瘤的临床表现、影像学特征及病理学特点，以期减少眼眶淋巴瘤的误诊，提高生存率。方法：对2020年1月至2020年12月中山大学中山眼科中心诊治的71例眼眶MALT结外边缘区B细胞淋巴瘤的临床、影像及病理学资料进行回顾性分析。结果：71例患者中，男38例，女33例；左侧眼眶31例，右侧眼眶34例，双侧眼眶6例；原发病例67例，复发病例4例；年龄23~84岁，病程为3个月~15年。最常见的临床表现是眼部肿块和不同程度的眼球活动受限；磁共振成像(magnetic resonance imaging，MRI)检查见密度均匀的软组织影，呈“铸造样”，眼球内未见侵犯；组织病理检查可见肿瘤由小至中等大小淋巴样细胞组成，瘤细胞呈弥漫或片状生长，核小到中等、不规则，核仁不明显，部分细胞呈单核样淋巴瘤细胞改变，其中9例可见浆细胞样分化，伴浆细胞分化的病例kappa与lambda的表达不对称。结论：眼眶MALT结外边缘区B细胞淋巴瘤有多种临床表现，影像学检查具有一定的特征，可辅助术前诊断。病理学检查可用于术后的准确诊断及分型，据此制定合适的治疗方案，提高疗效。

Objective: To investigate the clinical, imaging and pathological features of orbital extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT), so as to reduce misdiagnosis and improve survival rate.Methods: The clinical, imaging and histopathological data of 71 patients with orbital extranodal marginal zone B cell lymphoma of MALT who were treated in Zhongshan Ophthalmic Center, Sun Yat-sen University from Jan.2020 to Dec. 2020 were retrospectively analyzed. Results: There were 38 males and 33 females in the 71 patients.There were 31 cases located on the left orbit, 34 cases on the right orbit, and 6 cases were bilateral; there were 67 primary cases and 4 relapsed cases. The age ranged from 23 to 84 years old. The disease course ranged from 3 months to 15 years. The main clinical manifestations included ocular masses and limited eyeball movement.Magnetic resonance imaging showed a “cast-like” soft tissue shadow of uniform density, and no intraocular invasion is observed. Histopathological examination showed that the tumor was composed of small to medium sized lymphocyte-like cells. Tumor cells exhibited diffuse or lamellar growth, with small to medium-sized irregular nuclei, and the nucleoli were inconspicuous. Some of the lymphocyte-like cells showed mononuclear lymphoma cells changes, and plasmacytoid differentiation was observed in 9 cases, in which kappa and lambda expressions were asymmetric.Conclusion: The clinical manifestations of orbital extranodal marginal zone B cell lymphoma of MALT are various, and imaging examination has certain characteristics, which can assist preoperative diagnosis.Pathological examination can be used for accurate diagnosis and classification after operation, based on which appropriate treatment plan can be made and curative effect can be improved.

眼附属器淋巴组织增生性疾病作为一类疾病的总称，包括了良性淋巴组织增生、非典型性淋巴组织增生、IgG4相关眼病以及多种恶性淋巴瘤在内的数十种疾病类型。临床诊断此类疾病应将患者眼部体征、影像学检查与病理学检查紧密结合。随着免疫表型及分子病理等检测技术的进步，此类疾病之间的鉴别诊断正逐渐清晰。本文就眼附属器淋巴组织增生性疾病进行系统性描述，并重点探讨该类疾病的病理鉴别诊断。

Ocular adnexal lymphoproliferative disease, as a general term, contains reactive lymphoid hyperplasia, atypical lymphoid hyperplasia, IgG4 related ocular disease and malignant lymphoma. The clinical diagnosis of this kind of disease should integrate patient’s symptoms, imaging features and pathology characteristics. Development of immunophenotyping, molecular pathology and other detection technology will help with the differential diagnosis of ocular adnexal lymphoproliferative disease. This article is going to discuss the etiology, epidemiology,diagnosis and treatment of ocular adnexal lymphoproliferative disease, with a focus on the clinicopathological differential diagnosis of such disease.

线状皮脂腺痣综合征(linear nevus sebaceous syndrome，LNSS)是一种以皮脂腺痣(nevus sebaceous，NS)为特征性改变，同时合并癫痫、智力迟钝、神经缺陷或骨骼畸形等病变的疾病。本文报道1例经病理组织学检查确诊的LNSS患者，同时伴有双眼脉络膜骨瘤和脑部先天发育异常。由于线状皮脂腺综合征伴双眼多发异常较为少见，本文将总结该例患者的临床和病理表现，旨在为临床诊疗提供一定参考资料。

Linear nevus sebaceous syndrome (LNSS) is a disease characterized by nevus sebaceous (NS) and accompanied by epilepsy, mental retardation, nerve defect or skeletal deformity. We report a case of linear sebaceous nevus syndrome diagnosed by histopathological examination with bilateral choroidal osteoma and congenital developmental abnormalities of the brain. Since linear sebaceous gland syndrome with binocular abnormalities is relatively rare, this paper will summarize the clinical and pathological manifestations of this patient, aiming to provide certain reference for clinical diagnosis and treatment.

目的：制备抗水通道蛋白4(aquaporin 4，AQP4)单克隆抗体，鉴定其免疫学性能为后期临床应用奠定基础。方法：合成AQP4优势抗原表位多肽，经过免疫小鼠、细胞融合及亚克隆筛选，制备可分泌高效价高亲和力的单克隆抗体细胞株，后期获取大量单克隆抗体通过酶联免疫吸附和免疫组织化学实验进行初步实验应用。结果：获得4株杂交瘤细胞株，均能稳定分泌高亲和力抗体，经酶联免疫吸附测定实验和免疫组织化学实验证实均能特异性识别人的AQP4蛋白。结论：成功制备了亲和力高、特异性强的抗人AQP4优势抗原表位的单克隆抗体，为小分子抗体以及抗体人源化制备奠定了基础，进而为治疗视神经脊髓炎相关疾病提供理论依据和技术支持。

Objective: To lay the foundation for clinical applications in the future, we prepare and identify the immunological properties of aquaporin 4 (AQP4) monoclonal antibodies. Methods: The dominant epitope polypeptides of AQP4 were synthesized and used to obtain the cell lines which secrete high tiler and high affinity monoclonal antibodies by immunizing BALB/c mouse cell fusion and screening of subclone. The large amount of monoclonal antibodies were obtained and used for practice via immunohistochemical staining and Enzyme-linked immunosorbent assay (ELISA). Results: Four hybridomas that can stably secret anti-AQP4 antibodies were obtained. ELISA, Western blot and Immunohistochemistry results suggested that the monoclonal antibodies specifically recognized human AQP4 protein.Conclusion: The anti-human AQP4 monoclonal antibody with high affinity and specificity was successfully generated, which thereby provides the foundation for the preparation of small molecule antibodies or humanized antibodies, and then lays a theoretical and technical basis for the treatment of neuromyelitis optica (NMO).

目的：分析各种病因摘除眼球的患儿中视网膜母细胞瘤(retinoblastoma，RB)的误诊率，并总结其临床和病理特点。方法：回顾性分析2003年以来在中山眼科中心因临床诊断为RB并接受眼球摘除的患者563例(577眼)，比较术前临床诊断和术后病理诊断，从中筛选出误诊病例，分析这些病例的临床和病理特点，包括患者年龄、性别、治疗过程、临床影像学、肿瘤病理分期、病理诊断以及分化程度。结果：共发现误诊病例22例(22眼)，误诊率3.91%，所有误诊病例均为单眼摘除患者。常见的临床误诊类型为：与Coats病相混淆12例(12眼)，与眼内炎症反应混淆5例(5眼)，以及与眼内其他肿瘤混淆5例(5眼)。所有患者中，>4岁者55例，其中误诊12例，误诊率21.82%。结论：与RB相关的临床误诊并不罕见，其中最常见的误诊病因是Coats病，>4岁患者是误诊的主要人群。

Objective: To investigate the misdiagnosis rate in enucleated eyes for retinoblastoma (RB), analyze the clinicopathological features and summarize differential diagnosis. Methods: Retrospective analysis was performed on 563 cases (577 eye) undergoing eyeball enucleation in Zhongshan Ophthalmic Center Sun Yat-sen University since 2003. Misdiagnosed cases were screened out by comparing the preoperative clinical diagnosis and postoperative pathological diagnosis. The clinical and pathological features of those misdiagnosed caseswere summarized, including age, gender, therapeutic process, imagological examination, pathological stage and pathological diagnosis. Results: Twenty-two cases had been misdiagnosed, which account for 3.91% of 563 enucleated eyeball cases. All of misdiagnosed cases had underwent monocular eyeball enucleation. Diseases that were easily misdiagnosed with RB were Coats disease (12/22), endophthalmitis (5/22) and other intraocular tumor (5/22). Among all patients, 55 patients were older than 4 years old, of which 12 cases were misdiagnosed,with a misdiagnosis rate of 21.82%. Conclusion: It is not unusual for clinical misdiagnosis of RB. Coats disease is the most frequent cause of RB misdiagnosis. Misdiagnosis rates is higher in patients over 4 years old.

目的：比较活体共聚焦显微镜和病理检查在角膜后部真菌感染的诊断阳性率，探讨两种检查方法在角膜后部真菌感染诊断中的价值。方法：回顾性病例对照研究。收集2009年11月至2020年12月在青岛眼科医院就诊并进行穿透性角膜移植手术治疗角膜后部真菌感染患者，术前均进行角膜刮片KOH涂片检查和活体共聚焦显微镜检查，术后病变角膜进行病理组织切片、过碘酸-Schiff法(PAS)染色和六亚甲基四胺银法(GMS)染色检查，比较不同检查方法诊断的阳性率。结果：18例角膜后部真菌感染患者角膜刮片KOH涂片均未检查到真菌菌丝，其中有16例患者经活体共聚焦显微镜检查到真菌菌丝(88.9%)，而2例患者在术前活体共聚焦显微镜检查中未查到病原体。术后病理检查PAS染色联合GMS染色，18例患者中18例均可检查到真菌菌丝，角膜后部真菌感染患者病理切片中可见角膜深基质层变性坏死，大量炎症细胞浸润，PAS染色和GMS染色可见典型真菌菌丝侵犯角膜基质深层，而角膜基质浅层及上皮层均未查见真菌菌丝。结论：活体共聚焦显微镜诊断角膜后部真菌感染具有一定的局限性，联合术后病理组织切片和特殊染色检查有助于提高角膜后部真菌感染的诊断率。

Objective: To compare the diagnostic rate between in vivo confocal microscopy and pathological examination in retrocorneal fungal infection. Methods: It is a retrospective study. A total of 18 patients with retrocorneal fungal infection and received PKP surgery in the Qingdao Eye Hospital from November 2009 to December 2020 were enrolled. KOH smear and in vivo confocal microscopy examination were performed before surgery, and pathological examination including periodic acid-schiff (PAS) stain and Grocott Methenamine Silver (GMS) stain were performed after surgery. Patients were diagnosed retrocorneal fungal infection based on in vivo confocal microscopy and pathological examination. The diagnostic rates of the two methods were compared. Results: None of the 18 patients with posterior corneal fungal infection were found to have fungal hyphae in the corneal smear.Sixteen patients (88.9%) were found fungal hyphae by in vivo confocal microscopy. Corneal stroma necrosis and a large number of inflammatory cells were shown by postoperative pathologic examination, and all patients were found fungal hyphae in posterior corneal stroma with PAS stain and GMS stain. Conclusion: Confocal microscopy has unique advantages such as non-invasive and rapid examination in the diagnosis of fungal keratitis.However, it needs to combine with pathological examination for diagnosing the retrocorneal fungal infection.

目的：研究泪腺腺样囊性癌不同病理组织学分型和分级间骨质破坏、神经侵犯和预后的差异。方法：回顾性分析2010年4月至2019年4月首都医科大学附属北京同仁医院眼肿瘤科收治并经病理组织学检测确诊的30例泪腺腺样囊性癌患者的完整病历资料。分析患者的一般病历资料、医学影像学资料、病理组织学检查结果以及预后情况等，并系统研究泪腺腺样囊性癌病理组织学分型和分级与患者预后的关系。结果：30例患者中，病理组织学分型为筛状型9例(30.0%)，实体型10例(33.3%)，混合型11例(36.7%)，其中实性和腺管混合型4例(13.3%)、实性和筛状混合型4例(13.3%)、筛状和腺管混合型3例(10.0%)。病理组织学分级为I级12例(40.0%)；II级4例(13.3%)；III级14例(46.7%)。不同病理组织学分型肿瘤骨质破坏发生率差异有统计学意义(P=0.046)；不同病理组织学分级肿瘤骨质破坏发生率差异无统计学意义(P=0.513)。不同病理组织学分型与分级肿瘤神经侵犯、远处转移发生均无相关性(均P>0.05)。在行手术联合放射治疗后，不同病理组织学分型肿瘤的无复发率差异有统计学意义(P<0.05)；实体型的无复发率在36个月内最低(P=0.037)；而病理学分级与手术联合放射治疗后的无复发率无关(P=0.059)。结论：泪腺腺样囊性癌病理组织学分型与骨质破坏发生率和复发率显著相关，其中实体型肿瘤较易复发。而病理学分型与神经侵犯和远处转移发生率无关。病理组织学分级与骨质破坏、神经侵犯、远处转移和复发率无显著相关性。

Objective: To investigate the differences in bone destruction, nerve invasion, and prognosis of lacrimal gland adenoid cystic carcinoma (LGACC) among different histologic types and grades. Methods: A retrospective analysis was performed on 30 cases of lacrimal gland adenoid cystic carcinoma confirmed by histopathology who were admitted to the Department of Ophthalmic Oncology, Beijing Tongren Hospital, Capital Medical University from April 2010 to April 2019. The general data, imaging findings, histological examination and prognosis were collected and analyzed, with the focus on the relationship between the histological characteristics and the prognosis of lacrimal gland adenoid cystic carcinoma. Results: Among the 30 patients, 9 cases of cribriform type (30.0%); 10 cases of solid type (33.3%); 11 cases of mixed type (36.7%), including 4 cases of solid and glandular (13.3%), 4 cases of solid and cribriform (13.3%), 3 cases of cribriform and glandular (10.0%). The histopathological grade was I in 12 cases (40.0%), II in 4 cases (13.3%) and III in 14 cases (46.7%). There was statistical difference in the incidence of bone destruction among different histological types (P=0.046). There was no significant difference in the incidence of bone destruction among different histological grades (P=0.513).There was no significant difference between different histological grades and types and the incidence of nerve invasion and distant metastasis (all P>0.05). After surgery combined with radiotherapy, there was a statistical difference in the recurrence-free rate of different histological types, and the recurrence-free rate of solid type was the lowest within 36 months (P=0.037). Histological grade was not associated with recurrence-free rate after surgery combined with radiotherapy (P=0.059). Conclusion: The histological type of adenoid cystic carcinoma of lacrimal gland was significantly correlated with the incidence of bone destruction and recurrence rate, in which solid type was more likely to relapse. And the histological type was not associated with the incidence of nerve invasion or distant metastasis. Histopathological grade was not significantly associated with the rate of bone destruction, nerve invasion, distant metastasis, and recurrence.

目的：探讨眼眶原发性滑膜肉瘤(synovial sarcoma，SS)的临床病理学及分子遗传学特点。方法：收集1例复旦大学附属眼耳鼻喉科医院眼科2020年10月收治并经病理学检查证实为眼眶原发性SS的病例，同时回顾性分析文献中已报道的10例眼眶原发性SS的临床及病理检查资料，包括临床表现、影像学检查、组织学特点、免疫表型及分子病理学检查结果。结果：患者女，53岁，因“复发性右眼眶内肿物13余年”收治入院。SS组织病理学：肿瘤由弥漫分布的单一短梭形细胞组成，肿瘤细胞异型性明显，胞质少，核分裂多见；肿瘤侵犯结膜下、巩膜表面、视神经鞘膜、眶内肌肉及纤维脂肪组织。免疫组织化学检查提示波形蛋白(Vimentin)、Calpolnin、CD99、Bcl-2均阳，SMARCB1(INI-1)部分阳/弱阳。荧光原位杂交(fluorescence in situ hybridization，FISH)法检测到SS18基因易位。回顾性总结文献中已报道的10例和本例(总共11例)眼眶SS患者，其中男性2例，女性9例，左眼6例，右眼5例；患者发病年龄为1~53岁，平均年龄22岁，中位年龄24岁。患者术前病程范围较广，为1周~13年。11例中，5例症状至少出现3年以上，多表现为进行性眼球突出伴眼球移位及运动受限，疼痛及视力下降。CT和MRI上多表现为分叶状或者卵圆形软组织肿块，部分因出血坏死出现囊性外观，增强扫描显示病灶呈不均匀强化。组织学上，本组11例眼眶SS中单相纤维型7例，双相型4例，单相纤维型中有2例存在分化差的成分。免疫组织化学染色显示：上皮样成分表达上皮标记(CKpan、CK7、CK19)和Vimentin；梭形细胞表达Vimentin、CD99、Bcl-2、Calpolnin、TLE1及灶性表达上皮标记。结论：眼眶原发性滑膜肉瘤罕见，形态上需要和眼眶其他软组织来源恶性肿瘤相鉴别，其具有特征性t(x:18)(p11;q11)染色体易位，产生SY T-SSX融合基因，分子病理学的检测有助于最后确诊。

Objective: To investigate the clinicopathological and molecular genetics features of synovial sarcoma (SS) of the orbit. Methods: We retrospectively reviewed 10 published cases of primary SS of the orbit, along with 1 case of primary SS of the orbit confirmed by pathology who was admitted to the ophthalmology department of Eye & ENT Hospital of Fudan University in October 2020. The clinical data, radiological findings,morphology, immunophenotype and genetic characteristics of the cases were analyzed. Results: Our case was a 53-year-old woman with an SS in the right orbit, which had recurred multiple times. Histopathologic examination showed a primitive tumor composed of spindled and ovoid cells. Focal infiltration was observed in adjacent structures, such as the sub-conjunctiva, scleral surface, optic nerve sheath, muscle, and fibro-fatty tissue. Immunohistochemistry showed positivity for vimentin, calponin, CD99, and Bcl-2 and loss of INI-1expression, which is typical of SS. Fluorescence in situ hybridization (FISH) showed the (X;18)translocation in the tumor cells. The analysis included 2 males and 9 females aged between 1 and 53 years old (mean: 22 years; median: 24 years). Among the SS cases, 6 left eyes and 5 right eyes (all monocular cases)were affected. Symptoms had been present from 1 week to 13 years in the case from our hospital, while in 5 cases, symptoms had been present for at least 3 years. Common clinical features of the patients included proptosis or globe displacement, decreased vision, and pain. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed an ovoid mass with heterogenous enhancement and a cystic appearance,which was probably attributable to hemorrhage or necrosis. Of these 11 cases, 7 cases were biphasic SS,4 were monophasic fibrous SS, and 2 were poorly differentiated in monophasic SS. Immunohistochemistry revealed positivity for pan-cytokeratin (CKpan), CK7, CK19, vimentin, cluster of differentiation 99 (CD99),B-cell lymphoma 2 (Bcl-2), calponin and transducin-like enhancer protein 1 (TLE1). Conclusion: Primary SS of the orbit is extremely rare and needs to be distinguished from other spindle cell tumors of orbital soft tissue. The SS diagnosis is based on the presence of the t(X;18) (p11; q11) translocation, which results in an SYT-SSX fusion gene.