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.
眼内淋巴瘤(intraocular lymphoma，IOL)比较罕见。按起源位置分为两种类型，主要类型为原发性眼内淋巴瘤(primary intraocular lymphoma，PIOL)，也称为原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma，PCNSL)；另外一种类型为继发性眼内淋巴瘤(secondary intraocular lymphoma，SIOL)，为中枢神经系统以外的淋巴瘤转移至眼内。按肿瘤类型主要分为三类，主要类型为眼内弥漫大B细胞淋巴瘤，属于高级别淋巴瘤，预后较差；其次为少见的主要侵犯脉络膜的黏膜相关淋巴组织结外边缘区B细胞淋巴瘤，属于低级别淋巴瘤，预后较好；第三种类型为极少见的眼内NK/T细胞淋巴瘤，属于高级别淋巴瘤，预后极差。该病的诊断对眼科医生和病理医生都极具挑战性。实验室检测方法主要包括病理学、免疫细胞化学、流式细胞术、细胞因子及基因重排等，但眼内病理活检仍然是该病诊断的金标准。该病的治疗主要为眼内局部化疗、放射治疗及系统性化疗。IOL早期常因误诊而耽误治疗，目前该病明确诊断时多在患者出现症状后4~40个月，多数病例早期被误诊为葡萄膜炎而失去治疗的最佳时机，导致预后较差。因此应充分认识IOL的早期表现，早期诊断、早期治疗，从而大大提高疗效。
Intraocular lymphomas (IOL) are rare malignant neoplasms including primary intraocular lymphoma (PIOL) and secondary intraocular lymphoma (SIOL). The former is also known as primary central nervous system lymphoma(PCNSL). The latter is a kind of lymphoma metastasizing to the eye from outside the central nervous system. IOL can further be divided into three different types. The most common type is vitreoretinal high-grade diffuse large B-cell lymphoma with poor prognosis. The less common type is primary choroidal extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue, which is low-grade B-cell lymphoma with better prognosis.The rare type is NK/T cell lymphomas with very poor prognosis. The diagnosis of this disease is challenging for both ophthalmologists and pathologists. Laboratory testing methods mainly include cytology/pathology,immunocytochemistry, flow cytometry, cytokine analysis and gene rearrangement detection. The detection of malignant lymphoid cells cytologically/pathologically is still the gold standard for diagnosing IOL. The treatment involves local chemotherapy, radiotherapy and systemic chemotherapy. Most intraocular lymphomas at early stage are misdiagnosed as uveitis and proper treatment is often delayed with poor diagnosis due to the lost of best time for treatment. So far, the delay between the diagnosis and the onset of ocular symptoms ranges from 4 to 40 months. Therefore, we should fully understand the early manifestations of intraocular lymphoma and early diagnose and timely treat the disease in order to improve prognosis.
目的：探索用细胞块制备试剂盒对眼内玻璃体液微量细胞制备细胞块的成功率，苏木素-伊红(hematoxylin-eosin，HE)染色效果及技术要点。方法：收集中山大学中山眼科中心临床病理科2020年9月至2021年1月由临床送检的25例玻璃体液(含玻璃体切割液)，应用细胞块制备试剂盒制备细胞块后，常规固定、脱水、包埋、切片，随后进行HE染色，观察染色效果。结果：25例玻璃体细胞蜡块制作成功率达到100%，制片后HE染色效果好，背景干净，细胞形态清晰，核质对比分明。结论：应用细胞块制备试剂盒能将眼内玻璃体液微量细胞制成蜡块，极大提高了标本的利用率，为后续的病理研究提供丰富的材料。
Objective: To explore the effect and technical key points of the cell block preparation kit for collecting a few cells in ocular vitreous humor. Methods: A total of 25 cases of vitreous humor (including vitrectomy fluid) were collected from Zhongshan Ophthalmic Center, Sun Yat-sen University from September 2020 to January 2021.Cell block preparation kit was used to prepare cell blocks, which were routinely fixed, dehydrated, embedded,sectioned, and then hematoxylin-eosin (HE) stained. Results: The success rate of 25 cases of vitreous cell paraffinblocks reached 100%, and the morphology of the cells was clear with clean background and shape contrast of nucleus and plasma in HE staining. Conclusion: The cell block preparation kit can make the cells of intraocular vitreous humor into paraffin blocks, which greatly improves the utilization rate of specimens and is conducive to providing abundant materials for pathological studies.
睫状体髓上皮瘤是一种源自神经上皮层的恶性肿瘤，易因其伪装特性而被漏诊和误诊。本文报道了1例3岁9个月的男性患儿，眼部表现似晶状体破裂，经部分肿物切除活检，病理诊断为睫状体恶性髓上皮瘤。
Medulloepithelioma of the ciliary body is a kind of malignant tumor which arises from neuroepithelium. It is easily misdiagnosed or miss diagnosed due to its masquerade feature in the clinical practice. We report a boy with a tumor in his right eye at age of 3 years and 9 months presented first with lens rupture. Diagnosis of malignant medulloepithelioma of ciliary body was made pathologically by biopsy of the partial tumor.
睫状体髓上皮瘤是一种源自神经上皮层的恶性肿瘤，易因其伪装特性而被漏诊和误诊。本文报道了1例3岁9个月的男性患儿，眼部表现似晶状体破裂，经部分肿物切除活检，病理诊断为睫状体恶性髓上皮瘤。
Medulloepithelioma of the ciliary body is a kind of malignant tumor which arises from neuroepithelium. It is easily misdiagnosed or miss diagnosed due to its masquerade feature in the clinical practice. We report a boy with a tumor in his right eye at age of 3 years and 9 months presented first with lens rupture. Diagnosis of malignant medulloepithelioma of ciliary body was made pathologically by biopsy of the partial tumor.
