Merkel细胞癌(Merkel cell carcinoma，MCC)是一种少见的高度恶性的皮肤原发性神经内分泌癌，发生于眼睑的MCC更加罕见。本文对2例眼睑MCC患者的临床病史和治疗过程进行回顾和总结。1例初发患者经手术彻底切除，效果良好，随访7年，未再复发；1例患者在外院切除术后2个月，于眼睑原位复发，再次行手术彻底切除，2个月后同侧腮腺淋巴结及颈前淋巴结扩散，目前仍在肿瘤科进一步放化疗中。复习相关文献并结合本组病例提示，MCC诊断主要依靠病理检查，彻底切除病灶并结合术后放化疗是其治疗的主要手段，免疫治疗是目前新的发展趋势。

The Merkel cell carcinoma (MCC) is a rare highly malignant primary neuroendocrine carcinoma of the skin,especially in the eyelids. In this report, the clinical history and treatment course of 2 patients with MCC of the eyelid were reviewed and summarized. Patient 1 with primary MCC of eyelid was treated with complete surgical excision, with good results. Follow-up for 7 years had shown no recurrence. Patient 2 with the eyelid relapse in situ two months after resection in another hospital, was treated with complete surgical excision again, but the tumor metastasized to the ipsilateral lymphonodi parotidici and cervical lymph nodes two months after the surgery. The patient is now going through radiotherapy and chemotherapy in oncology department. According to the review of literatures, the diagnosis was based on the pathologic evidence. Complete removal of lesion combined with postoperative radiotherapy and chemotherapy is a major means of treatment for MCC. Immunotherapy will play a more important role in the future.

眼眶泪腺淋巴上皮癌是一种非常少见的恶性肿瘤。本例为一位46岁女性患者，表现为左眼睑红肿、眼球突出和视力下降，临床和影像学表现类似于泪腺炎型假瘤。手术完整切除后送检病理检查，其特征是未分化的癌巢之间有大量分化成熟的淋巴细胞、浆细胞浸润，确诊为淋巴上皮癌。术后行化学治疗和局部放射治疗，随访6.5年无复发。眼眶泪腺淋巴上皮癌的临床表现容易与泪腺炎型假瘤相混淆，应该进行病理学检查以明确其恶性表型，完整切除后辅助放化学治疗有较好的效果。

Lymphoepithelial carcinoma of the orbital lacrimal gland is a rare carcinoma. A 46-year-old female patient presented with red and swollen left eyelid, proptosis and blurred vision. The clinical and imaging manifestations were similar to inflammatory pseudotumor. Pathological examination was performed after complete resection.Pathological features showed undifferentiated carcinoma nests surrounded by obvious infiltration of non-neoplastic lymphocytes and plasma cells, which was consistent with the diagnosis of lymphoepithelial carcinoma.Chemotherapy and local radiotherapy were performed after the resection. The case did not relapse after 5 years of follow-up. The clinical manifestations of orbital lacrimal lymphoepithelial carcinoma were easily confused with inflammatory pseudotumor. Pathological examination should be performed to clarify its malignant phenotype.Adjuvant radiotherapy and chemotherapy after complete resection had a good effect.

目的：采用角膜激光共焦显微镜观察研究颗粒状角膜营养不良家系患者的角膜状况。方法：采用角膜激光共焦显微镜对4例(6只眼)颗粒状角膜营养不良患者的角膜进行扫描检测，对比裂隙灯及普通显微镜观察分析检查情况。结果：颗粒状角膜营养不良患者的病变角膜裂隙灯下呈现面包屑样的灰白色混浊，在苏木精-伊红(hematoxylin-eosin，HE)染色中呈现出伊红染的颗粒，沉积于基质板层之间，在共焦显微镜下则为中高反光的团块，相互融合，并取代了基质细胞。结论：颗粒状角膜营养不良患者角膜病变从裂隙灯到普通HE染色下的显微镜，乃至高倍的共焦显微镜观察到的沉积物，分布情况相吻合，印证角膜病变发展的状况，有利于了解该病变的发展情况。

Objective: To observe and study the corneal in patients of family with granular corneal dystrophy by confocal laser scanning microscopy. Methods: The corneas of 4 patients (6 eyes) with granular corneal dystrophy were scanned by laser confocal microscopy, and the results were compared with those observed by slit lamp and ordinary microscope. Results: The image were gray and white bread-crumb-like opacity under the slit-lamp of cornea in patients of family with granular corneal dystrophy, were eosin-stained granules deposited between stroma layers in hematoxylin-eosin (HE) stain under the microscopy, were high reflective masses under the confocal laser scanning microscopy, which fused with each other. Conclusion: The image of the corneal in patients of family with granular corneal dystrophy were clearer and clearer by from Slit Lamp and microscopy to the confocal laser scanning microscopy. The distribution of sediments was consistent with each other. It can be a good understanding of the development of the disease.

目的：探讨血管生成拟态(vasculogenic mimicry，VM)与翼状胬肉初发型及复发型的相关性。方法：采用血小板-内皮细胞黏附分子/过碘酸雪夫(platelet endothelial cell adhesion molecule-1/periodic acid-schiff，CD31/PAS)免疫组织化学双重染色法检测139例翼状胬肉组织(初发型105例；复发型34例)和10例正常结膜中VM的表达，分析VM与初发型及复发型翼状胬肉的相关性及其与患者性别、年龄等因素的关系。原代培养人翼状胬肉成纤维细胞(human pterygium fibroblasts，HPFs)，免疫细胞化学染色法鉴定，利用三维培养及PAS染色观察初发型和复发型HPFs构成VM管腔个数的差异。结果：10例正常结膜均未见VM结构，初发型翼状胬肉VM阳性率43.81%，复发型翼状胬肉VM阳性率82.35%，差异具有统计学意义(P<0.001)。相关性分析显示VM与复发型翼状胬肉呈显著正相关(r=0.332)。不同性别、年龄及病程的翼状胬肉患者VM的表达差异均无统计学意义(均P>0.05)。原代培养的HPFs Vimentin表达阳性，符合成纤维细胞特性。细胞三维培养及PAS染色结果提示HPFs具有构建体外VM模型的能力，且复发型HPFs构成的VM管腔数明显高于初发型，差异具有统计学意义(P<0.01)。结论：翼状胬肉组织中存在VM结构，可作为其血供途径之一。VM与翼状胬肉的复发具有密切关系。

Objective: The purpose of this study was to investigate the correlation of vasculogenic mimicry in the primary and recurrent pterygium. Methods: Platelet endothelial cell adhesion molecule-1/periodic acid-schiff (CD31/PAS)immunohistochemical double staining method was adopted to detect the expression of VM in 139 cases of pterygium (105 cases of primary pterygium and 34 cases of recurrent pterygium)and 10 cases of normal conjunctival tissues. The correlation between VM and primary pterygium, recurrent pterygium and the factors such as gender and age of patients were analyzed. Human pterygium fibroblasts (HPFs) were primary cultured and identified by immunocytochemical staining. The differences in the number of VM channels between primary HPFs and recurrent HPFs were observed by three-dimensional culture and PAS staining. Results: There was no VM structure in 10 normal conjunctiva and the positive rate of VM was 43.81% in primary pterygium and 82.35% in recurrent pterygium with a significantly difference (P<0.001). Correlation analysis showed a significant positive correlation between VM and recurrent pterygium (r=0.332). There was no significant difference in the expression of VM in pterygium patients with different sex, age and course (all P>0.05). Vimentin was positive in the primary cultured cells, which was consistent with the characteristics of fibroblasts. The results of three-dimensional culture and PAS staining indicated that HPFs had the ability to construct VM model in vitro, and the number of VM channels constituted by recurrent HPFs was significantly higher than that by primary HPFs, the difference was statistically significant (P<0.01). Conclusion: VM exists in pterygium tissues, and it can be used as one of the blood supply routes, which is closely related to the recurrence of pterygium.

目的：分析发生于眶骨的占位性病变的CT表现及其与组织病理学之间的关系。方法：回顾性收集15例经临床表现、影像学特征、病理证实为发生于眶骨占位病变患者的临床资料，分析其CT检查、组织病理学切片及其他临床表现。结果：在15例患者中，经病理诊断为骨瘤的患者有4例，骨内血管瘤3例，骨样骨瘤和骨化性纤维瘤各2例，嗜酸性肉芽肿3例，尤文肉瘤1例。骨瘤由成熟板层骨构成，对应在CT上呈现为高密度的骨样结节状隆起；骨内血管瘤病理主要表现为骨小梁间的畸形血管，在CT上表现为典型的“栅栏状”“蜂窝状”特征；骨样骨瘤以骨样组织和结缔组织为主，在CT上为类圆形的高密度影，中央为典型的低密度“瘤巢“影，外周为增生的骨密度影；骨化性纤维瘤由增生的纤维组织及骨样组织构成，CT表现为肿瘤表面常有骨壳包绕，病变多呈圆形或椭圆形；嗜酸性肉芽肿主要由大量朗格汉斯细胞组成，对应CT上的软组织肿块影，可伴溶骨性破坏；尤文肉瘤以低分化的小圆细胞为主，CT表现为虫蚀样骨质破坏，破坏的骨组织间有软组织病变。结论：发生于眶骨的各类病变有其特有的病理组成，不同的病理组成在CT上表现出相应的影像特征。

Objective: To analyze computed tomography (CT) manifestations of space-occupying lesions of the orbital bone and their relationship with histopathology. Methods: The clinical data of 15 patients with orbital bone occupying lesions confirmed by clinical manifestations , imaging features and pathological symptoms features were retrospectively collected, and their CT examination, histopathological sections and other clinical manifestations were analyzed. Results: Among the 15 patients, 4 cases were pathologically diagnosed with osteoma, 3 cases of intraosseous hemangioma, 2 cases of osteoid osteoma and 2 cases of ossifying fibroma, 3 cases of eosinophilic granuloma and 1 case of Ewing’s sarcoma. Osteoma was composed of mature lamellar bones, which corresponded to a high-density osteoid nodular protuberance on CT. The pathological features of intraosseous hemangioma were mainly malformed blood vessels between bone trabeculae and typical “fence” and “honeycomb” features on CT. Osteoid osteoma was mainly composed of bone-like tissue and connective tissue, mainly showing round high-density shadow, typical low-density “tumor nest” shadow in the center, and hypertrophic bone density shadow in the periphery. Ossifying fibroma was composed of proliferative fibrous tissue and bone-like tissue. CT showed that the surface of the tumor was often surrounded by bone shell, and the lesions were mostly round or oval. Eosinophilic granuloma was mainly composed of a large number of Langerhans cells, which corresponded to the soft tissue mass on CT and could be accompanied by osteolytic destruction. Ewing’s sarcoma was mainly composed of poorly differentiated small round cells, and CT manifestations were worm-eaten bone destruction with soft tissue lesions between destroyed bone tissues. Conclusion: Various lesions occurring in the orbital bone have their own special pathological composition. Different pathological components show corresponding imaging features on CT.

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.

黏膜相关淋巴组织(mucosa-associated lymphoid tissue lymphomas，MALT)淋巴瘤是原发性眼附属器淋巴瘤(primary ocular adnexal lymphoma，POAL)中最常见的病理类型。目前，原发性眼附属器黏膜相关淋巴组织淋巴瘤(primary ocular adnexal mucosa-associated lymphoid tissue lymphoma，POAML)的临床类型和临床表现尚未被眼科医师熟练掌握，临床治疗亦无共识和指南。本文根据POAML起源位置，重点介绍各临床类型的早中期临床表现，以及针对各临床类型和病变范围的个体化治疗方法。

Mucosa-associated lymphoid tissue lymphomas (MALT) lymphoma is the most common pathologic type in primary ocular adnexal lymphoma (POAL). Currently, the clinical types and manifestations of primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (POAML) have not been well understood by ophthalmologists, and there is no consensus or guideline for clinical treatment. According to the original location, this paper focuses on the clinical manifestations of early and intermediate-stage POAML, as well as the individualized treatment for each clinical type and lesion range.

近年来日益强大的眼内液分子/细胞生物学检测技术因其简便、快捷和高效的特点，使得眼科医生在诊断眼内淋巴瘤时倾向于单纯只依据此类方法而淡化病理诊断的重要性。眼内液分子/细胞生物学技术因其本身只能“间接提示肿瘤细胞存在”的局限性而不能作为眼内淋巴瘤的确诊依据。眼内组织/细胞病理仍然是眼内淋巴瘤诊断的金标准，其价值和地位不能被其他任何分子/细胞生物学检测手段所替代。理解并掌握各种诊断、检测技术的优势和局限性，规范和优化眼内组织/细胞病理标本的采集、保存和送检流程有助于提高眼科临床医生对眼内淋巴瘤的诊断效率和医疗质量。

In recent years, more and more powerful molecular/cellular biological techniques of intraocular fluid have made ophthalmologists tend to only rely on these methods in the diagnosis of intraocular lymphoma because of their features of simplicity, fastness and efficiency. The molecular/cellular biological techniques of intraocular fluid cannot be used as the basis for the diagnosis of intraocular lymphoma because it can only indicate the existence of tumor cells indirectly. Intraocular tissue/cell pathology remains the gold standard for the diagnosis of intraocular lymphoma, and its importance cannot be replaced by any other molecular/cell biological methods. Understanding and mastering the advantages and limitations of various diagnostic techniques, standardizing and optimizing the collection, preservation and submission process of intraocular tissue/cell specimens will help ophthalmologists improve the diagnostic efficiency and medical quality of intraocular lymphoma.