Peripapillary choroidal neovascularization associated with melanocytoma of the optic disc: a clinicopathologic case report

Background Melanocytoma of the optic disc is a benign melanocytic tumor that rarely causes visual impairment. We report a case of a melanocytoma of the optic disc with a decreased vision related to a peripapillary choroidal neovascular membrane (PCNVM) that was successfully treated by submacular surgery.Methods A 45-year-old southern European patient had a melanocytoma of the optic disc in his left eye with vision of 20/100. Fluorescein angiography demonstrated a PCNVM impeding the fovea associated with a subretinal hemorrhage.Results The patient underwent a complete vitrectomy and removal of the PCNVM. Subsequently, the subretinal hemorrhage disappeared and visual acuity improved to 20/25. Visual acuity remained good for a period of 14 months’ follow-up without any recurrence of neovascular membrane.Conclusions Submacular surgery is a potentially effective treatment for large PCNVM associated with a melanocytoma of the optic disc.     

Successful treatment of melanocytoma associated choroidal neovascular membrane with intravitreal bevacizumab

Melanocytoma of the optic disc is a benign melanocytic tumour that rarely causes visual impairment. We report a rare case of choroidal neovascularization (CNV) in association with optic disc melanocytoma and its response to intravitreal injection of the anti-vascular endothelial growth factor (VEGF), bevacizumab. The choroidal neovascular membrane regressed following a single intravitreal bevacizumab injection with formation of a scar. CNV associated with optic disc melanocytoma is rare. Intravitreal anti-VEGF treatment may be an effective treatment for CNV associated with optic disc melanocytoma.     

脉络膜黑色素瘤组织病理学分析

目的 观察脉络膜黑色素瘤的组织病理学特征。 方法 回顾分析64例病理确诊的脉络膜黑色素瘤病理资料。按照美国眼黑色素瘤多中心研究组的测量方法和WHO的分类标准，测量和观察大体标本中的肿瘤大小和光学显微镜下的肿瘤细胞学类型；以肿瘤前缘累及部位对肿瘤所处位置进行分类；以肿瘤细胞向外浸润程度对肿瘤蔓延程度进行分级。 结果 64例脉络膜黑色素瘤中，大肿瘤25例，占39.1%；中等大小肿瘤31例，占48.4%；小肿瘤8例，占12.5%。梭形细胞型42例，占65.6%,其中，梭形细胞A型15例,占23.4%，梭形细胞B型27例,占42.3%;上皮样细胞型7例,占10.9%;混合型10例,占15.6%;其它型5例,占7.8%。肿瘤细胞未累及巩膜者25例,占39.1%;累及巩膜但限于巩膜层内者22例,占34.4%;穿透巩膜全层达眼球表面者12例,占18.7%;眶内浸润者5例,占7.8%。 结论 脉络膜黑色素瘤组织病理学特征变化多样，临床上以梭形细胞型最常见，易伴巩膜浸润。 (中华眼底病杂志, 2006, 22: 161-165)     

Idiopathic retinal gliosis mimicking a choroidal melanoma

A 37-year-old woman has followed for more than 10 years with an amelanotic peripheral intraocular tumor. This solitary lesion grew slowly and, because of the possibility of an amelanotic melanoma, the eye was enucleated. Histopathology revealed a localized "massive" retinal gliosis (MRG). Immunohistochemical staining for S-100 protein and glial fibrillary acidic protein (GFAP) were positive. Electron microscopy showed a highly fibrillar component and basement membrane formation in the extracellular space. Proliferating cells contained abundant fine filaments. These findings confirmed the glial origin of the proliferating cells. The previously undefined relationship of these localized forms of MRG with solitary astrocytomas is discussed.     

弥漫型脉络膜黑色素瘤的临床病理特点

目的 观察弥漫型脉络膜黑色素瘤临床病理特点。方法 回顾分析119例病理学检查确诊的脉络膜黑色素瘤患者中11例弥漫型脉络膜黑色素瘤患者的临床病理资料。患者中10例因视力丧失或眼痛就诊,1例外地医院诊断Coats病,继发性青光眼和眼球萎缩要求眼球摘除。临床诊断脉络膜肿物或黑色素瘤8例,绝对期青光眼2例,Coats病、眼球萎缩1例。眼球摘除9例,眼球摘除和眶内肿物切除2例。应用Ki-67免疫组织化学染色,检测细胞增生情况。结果 11例弥漫型脉络膜黑色素瘤均为基底广泛的扁平状肿物。肿瘤基底直径12～20 mm,厚度 2～4 mm。混合细胞型9例,上皮样细胞型1例,坏死型1例。瘤细胞侵犯巩膜7例、侵犯眶内3例,继发性青光眼7例。Ki-67阳性细胞7%～13%,平均阳性细胞9%,多分布于肿瘤基底部,上皮样瘤细胞的表达高于梭形瘤细胞。结论 弥漫型脉络膜黑色素瘤具有特殊生长方式,早期临床诊断困难,有些病例容易诊断为其它脉络膜肿物或青光眼。由于肿瘤基底广泛,容易侵犯眶内和发生转移,预后较差。     

X刀治疗脉络膜黑色素瘤的疗效评估

目的　观察X刀立体定向放射治疗对脉络膜黑色素瘤的疗效及贯穿眼睑直肌牵拉眼球固定法的效果。方法　采用Vrian 2 10 0C直线加速器 ,6× 10 6VX刀对 16例 (16只眼 )瘤体为 11 8mm× 10 0mm× 4 5mm的脉络膜黑色素瘤患者进行立体定向放射治疗 (平均边缘剂量为 2 3 4Gy) ,观察其临床疗效 ,并对其进行评估。放疗前先将眼球固定 ,即局部浸润性麻醉 (不用球后注射 ) ,于 4条直肌附着处置牵引缝线 ,上下穹窿中部贯穿眼睑 ,分别固定于眉上、眶下缘、鼻根、眶外缘处深及骨膜。平均随访 18 5个月 (12～ 36个月 )。结果　瘤体于治疗后 6～ 12个月内明显缩小 7例 (43 8% ) ;1～ 2年时 ,11例 (6 8 8% )肿瘤缩小。其中 4例肿瘤处残留小盘状痕迹 ,3例肿瘤缩小不明显 ,3例肿瘤因有轻微增大而行眼球摘除术。瘤体局部控制率为 81 3%。除视网膜及其膜下可见小片状出血外 ,余无其他严重并发症。眼球固定良好 ,放疗定位准确。结论　脉络膜黑色素瘤的X刀立体定向放射治疗法效果较好 ,无严重并发症 ,瘤体局部控制率高。贯穿眼睑直肌牵拉固定眼球法效果可靠     

Malignant melanoma arising in a choroidal magnacellular nevus (melanocytoma).

A case is presented of a 55-year-old Caucasian male whose right eye was enucleated for a mixed spindle-A and spindle-B malignant melanoma in 1967. The ophthalmoscopic picture, fluorescein angiography, and overlying peculiar orange pigmentation were suggestive of a malignant lesion. Study of serial sections of the entire lesion made possible the histologic reconstruction of the lesion, clinicopathologic correlation of the yellow-orange pigment, and the location of the nevus and mixed spindle-cell melanoma components.     

眼外蔓延的脉络膜黑色素瘤的临床观察

目的 总结眼外蔓延的脉络膜黑色素瘤的诊断方法和治疗经验.方法 回顾性系列病例研究.收集12例经病理学确诊的眼外蔓延期脉络膜黑色素瘤患者临床资料,总结病史特点、临床表现及影像学征象、治疗与随访结果.结果 4例既往有误诊为青光眼的病史;4例既往确诊者,其中3例接受肿瘤局部切除术.常见体征包括:视力下降,眼球突出,上睑下垂,眼球运动受限,结膜充血,眼压或眶压升高等,特异性体征为巩膜表面隆起性肿物.眼B超检查6例,典型表现为眼球壁结构中断,眼内、外肿瘤连续,眼外肿瘤呈低回声,或呈中、低回声混杂.CT检查6例,表现为肌锥内团块状肿瘤与球内肿瘤相连续,边清,均质,包绕眼球或视神经生长.所有患者行MRI T1和T2加权像检查,球内、外的肿瘤有4种类型的信号特征,肿瘤在T1加权像呈高信号,T2加权像呈低信号为典型表现.球外肿瘤的最大直径超过4 mm者有9例.MRI检查可显示球外多灶蔓延及远处转移,且有助于显示微小的球外蔓延灶,最小直径3 mm.11例患者行眶内容物剜除术,1例行眼球及球外瘤体摘除术.病理学检查显示肿瘤以上皮细胞型最为多见,有3种向眼外蔓延的途径.有随访记录者8例,术后联合化疗、放疗或干扰素治疗,随访1～5年,6例无眼部复发,全身情况良好,1例死亡,1例转移.结论 脉络膜黑色素瘤发展至眼外蔓延期的主要原因可能是延误治疗,临床缺少特异表现,确诊多需结合多种影像学检查.手术联合辅助治疗有助于减少局部复发和全身转移.

Abstract：

Objective To evaluate the clinical features, diagnostic methods and treatment of extraocular extension of choroidal melanoma. Methods It was a retrospective case series study. The records of 12 consecutive cases with extraocular extension of choroidal melanoma confirmed by pathologic examination were analyzed with special attention to the case histories, clinical manifestations, imaging findings, treatment and follow up results. Results Four patients were misdiagnosed as glaucoma. Another 4 patients were confirmed the diagnosis of melanoma and 3 of them received the operations of transscleral local resection. The common clinical manifestations included: impaired vision, exophthalmos,blephroptosis, limited ocular motility, conjunctival hyperemia, and increased ocular or orbital pressure, etc.Characteristic sign was raised mass on the surface of sclera. All cases underwent orbital MRI preoperatively,6 underwent ocular B-scan echography, and 6 underwent orbital CT scanning to evaluate for extraocular extension of tumor. Typical ultrasonography revealed the discontinued ocular wall and an intraocular mass with a continuous hypoechoic extraocular mass. In some cases the extraocular mass showed hypoechoic with medium echo area. CT scan showed a well-defined homogeneous intraconal mass connecting with intraocular lesion in 6 cases, which could infiltrate eye or optic nerve. The MR signal features of intraocular tumors with extraocular extension showed 4 patterns in all patients. The typical pattern was the tumor showed hyperintensity on T1 and hypointensity on T2-weighted image. The maximum diameter of extraocular tumor was measured over 4 mm in 9 cases. MRI was useful for demonstrating multiple extraocular lesions, remote metastatic lesion or micro extraocular tumor, of which minimal diameter was 3 mm in our cases. Orbital exenteration was performed in 11 cases and ocular enucleation with excision of extraocular tumor was in 1 cases. Microscopic examination showed the epithelioid-cell-type tumors were the most common. The tumor extended out of the eye by three types of way in our cases. Follow-up ranged from 1 to 5 years in 8 cases,who underwent chemotherapy, radiotherapy or interferon. Six cases had no recurrences and general health.Death and metastasis was respectively recorded in one case. Conclusions Delayed treatment could induce the growth of choroidal melanoma to the stage of extraocular extension, which has few specific clinical manifestations. The combination of multiple imaging examinations was helpful for the correct diagnosis.Surgical operation with adjunctive therapies could partly reduce recurrence and metastasis of melanoma.     

玻璃体视网膜联合手术切除脉络膜黑色素瘤

目的 探讨玻璃体视网膜手术切除脉络膜黑色素瘤的疗效、安全性及其适应证范围。 方法 8例脉络膜黑色素瘤,直径5～20　mm,厚4～12　mm。4例合并视网膜脱离。2例经眼内肿瘤切除,6例行板层巩膜脉络 膜切除。8例均联合玻璃体视网膜手术,包括玻璃体切割,过氟化碳液体应用,眼内光凝,气体或硅油充填。 结果 术后病理检查证实：7例为脉胳膜黑色素瘤,其中5例为梭形细胞型,2例混合细胞型,3例累及 睫状体。1例为脉络膜黑色素细胞瘤。经2～24个月(平均9.1个月)随访,未发现肿瘤复发与转移,眼球外形均无明显改变,5例视力在0.1以上。 结论 对选择性病例采用玻 璃体视网膜手术切除脉络膜黑色素瘤可以保留患眼,挽救视力,而且对切除组织病理检查可 明确诊断。（中华眼底病杂志,2000,16：139-212）     

脉络膜黑色素瘤眼球保留治疗结果初步观察

目的 观察保留眼球的治疗法治疗脉络膜黑色素瘤的临床效果。 方法 回顾分析44例脉络膜黑色素瘤患者44只患眼接受眼球保留治疗的临床资料，观察分析全身转移情况、眼球保留率以及视力预后。患者均为单眼发病，其中，以经瞳孔温热疗法（TTT）作为首选治疗者7只眼，占15.9%；以 106 Ru放射敷贴器近程放射治疗为首选治疗者25只眼，占56.8%；肿瘤切除联合 106 Ru放射敷贴器近程放射治疗者12只眼，占27.3%。治疗后随诊观察时间平均为13.3个月。 结果 44例患者在观察期内均未出现肿瘤全身转移。39例成功保留眼球，占88.6%。6只小肿瘤眼、14只中等大肿瘤眼和24只大肿瘤眼的眼球保留率分别为100%、92.9%和 83.3%。11只眼视力≥0.3，占28.2%；18只眼视力≥0.05~＜0.3，占46.2%；10只眼视力＜0.05，占25.6%。 结论 106 Ru放射敷贴器近程放射治疗和TTT治疗可以有效治疗中、小脉络膜黑色素瘤；部分大肿瘤选择肿瘤切除联合 106 Ru放射敷贴器近程放射治疗，其综合效果是否优于眼球摘除手术尚需进一步观察。 (中华眼底病杂志, 2006, 22: 150-153)     

Characteristics,management, and outcome of patients with uveal melanoma treated by Iodine-125 radioactive plaque therapy in a single tertiary cancer center in Jordan

Objective

To evaluate King Hussein Cancer Center experience in using Iodine-125 COMS radioactive plaque for treatment of Uveal Melanoma in Jordan.

高场磁共振对脉络膜黑色素瘤的诊断价值

目的评价高场磁共振（ＭＲＩ）对脉络膜黑色素瘤的诊断价值。方法采用ＧＥ１．５Ｔ磁共振扫描仪,对１２例黑色素瘤患者进行矢、冠、轴及斜位扫描,同时行脂肪抑制技术、增强扫描及病理检查。结果１２例黑色素瘤在Ｔ１ＷＩ均表现为不同程度的高信号,在Ｔ２ＷＩ上为低信号。其主要原因为黑色素瘤中的黑色素为顺磁性物质,可同时缩短Ｔ１和Ｔ２弛豫时间,以此可以同脉络膜其他病变相鉴别。１２例中有７例合并视网膜脱离。结论磁共振可在矢、冠、轴及斜位以小视野对眼眶进行扫描,并为临床治疗及手术准确定位;脉络膜黑色素瘤具有特殊的ＭＲＩ表现,可与脉络膜其他疾病鉴别     

广角激光扫描血管造影技术在脉络膜黑色素瘤诊断中的应用价值

目的 评价广角激光扫描血管造影技术在脉络膜黑色素瘤诊断中的应用价值。 方法 将24例临床诊断为脉络膜黑色素瘤的患者随机分为两组，分别应用激光扫描检眼镜的广角接触镜系统和非接触镜成像镜头对其行荧光素眼底血管造影和吲哚青绿血管造影的全景造影扫描，获得150°广角视野图像和30°视野图像资料，并对其成像质量进行综合评定。 结果 24例患者均获得满意的影像资料。广角成像系统提供了病变位置与其他解剖结构准确的毗邻关系，提供了病变的大小和造影过程中瘤体血管充盈情况的全面信息。同时将传统造影视野范围提高了3～5倍，有助于周边病变的筛查。 结论 广角激光扫描血管造影技术在脉络膜黑色素瘤诊断中有重要的应用价值。 (中华眼底病杂志, 2006, 22: 166-169)     

脉络膜黑色素瘤的磁共振成像分析及鉴别诊断

目的利用磁共振成像（ｍａｇｎｅｔｉｃｒｅｓｏｎａｎｃｅｉｍａｇｉｎｇ,ＭＲＩ）辅助诊断１２例脉络膜黑色素瘤,并做组织学检查。方法患者分别进行Ｂ超、ＣＴ和ＭＲＩ检查,对比分析三者的影像学特点。患眼均行手术摘除,并做病理检查。结果发现脉络膜黑色素瘤患者的ＭＲＩ图像：Ｔ１ＷＩ显示高信号,Ｔ２ＷＩ显示低信号特点。结论诊断脉络膜黑色素瘤除利用Ｂ超、ＣＴ外,ＭＲＩ检查亦为可靠的辅助诊断手段。     

脉络膜黑色素瘤敷贴放射治疗的初步观察

目的 观察脉络膜黑色素瘤敷贴放射治疗的效果。 方法 采用国产敷贴器对间接检眼镜、荧光素眼底血管造影、吲哚青绿血管造影、B型超声等综合检查确诊的脉络膜黑色素瘤患者21例21只眼进行治疗。棕色实性隆起的圆形或椭圆形肿物位于黄斑周围者7只眼、视盘周围者7只眼、血管弓及附近者5只眼、周边部者2只眼。B型超声检查显示瘤体最大径为13.0 mm×11.6 mm×9.6 mm。视力0.05及其以下者3只眼，0.06至0.2者4只眼，0.3及其以上者14只眼。敷贴器所用核素为 125 I，设定照射总量为100~120 Gy。其中肿瘤位于黄斑、视盘周围的14只眼同时合并采用经瞳孔温热疗法治疗。治疗后观察时间平均12个月，最长者达3年。以B型超声测量瘤体的基底及厚(高)度，若厚度超过原厚度的15%，或基底边缘大于原边缘250 mm视为肿物增大。 结果 治疗后视力下降9只眼，不变10只眼，提高2只眼。瘤体增大6只眼，不变12只眼，缩小3只眼。并发玻璃体积血2只眼，视网膜血管阻塞1只眼，视网膜分支静脉阻塞1只眼，黄斑皱褶1只眼，视网膜出血3只眼，部分性视神经萎缩3只眼，新生血管性青光眼1只眼，眼球摘出3只眼。 结论 用国产敷贴器对脉络膜黑色素瘤进行放射治疗具有确切的疗效。但对瘤体较厚、肿瘤位于黄斑或视盘旁者效果较差。 (中华眼底病杂志, 2006, 22: 157-160)     

人眼脉络膜黑色素瘤OCM-1细胞中肿瘤干细胞的分离纯化

目的分离及纯化人眼脉络膜黑色素瘤细胞OCM-1中的肿瘤干细胞。方法将OCM-1细胞复苏，使用Dubecco标准改良Eagle培养基（DMEM/F12）培养后，改用无血清化学限定培养基（SFM），并分离纯化培养肿瘤干细胞，用流式细胞仪检测神经干细胞特异性标记物CD133的表达比率,并对第六代细胞进行神经RNA结合蛋白（musashi-1）免疫细胞化学染色，显微镜下观察阳性细胞比率。结果在标准DMEM/F12培养基中贴壁生长的OCM 1细胞用SFM培养基培养后，贴壁细胞显著减少，至第6代时细胞均为悬浮团状集落生长，呈现典型的干细胞生长方式。在不同代的细胞中，CD133均有阳性，未经过分离的OCM-1细胞、经过SFM培养并分离的第1、2代OCM 1细胞的阳性比率分别2.5％、217％、57.8％，第6代的细胞CD133阳性比率达到79.8％，musashi-1强阳性表达。结论脉络膜黑色素瘤细胞中存在肿瘤干细胞。使用干细胞培养液培养，并通过限制分化、连续传代的方法，可分离纯化出悬浮克隆集落生长的干细胞。(中华眼底病杂志，2007， 23：87-90）     

眼内活组织检查技术诊断脉络膜肿瘤的临床价值

目的 探讨眼内活组织检查(活检)技术诊断脉络膜肿瘤的临床价值.方法 回顾性病例系列研究.对16例(16只眼)脉络膜肿瘤患者临床资料进行回顾性分析.患者术前均常规行裂隙灯显微镜、双目间接检眼镜、B超、彩色多普勒超声、双眼核磁共振、相干光断层扫描、荧光素眼底血管造影及吲哚氰绿脉络膜血管造影检查.所有患者均行标准三通道睫状体平坦部玻璃体手术,术中用玻璃体切割头切除并留取脉络膜肿瘤实质,合并玻璃体积血的患者留取玻璃体标本,肿瘤位于眼底赤道部及赤道部前的患者局部切除脉络膜肿瘤.活检标本、玻璃体标本及局部切除肿瘤标本送病理检查.对肿瘤活检标本与肿瘤局部切除标本的病理诊断进行一致性Kappa检验.结果 4例伴有玻璃体积血的患者行玻璃体标本活检,其中2例显示玻璃体有出血,2例找到脉络膜黑色素瘤的肿瘤细胞;16例脉络膜肿瘤活检标本经病理检查,其中阴性2例,脉络膜黑色素瘤11例,黑色素细胞瘤1例,局部出血2例,确诊率87.5%;12例肿瘤局部切除标本经病理检查,其中脉络膜黑色素瘤9例,黑色素细胞瘤1例,神经鞘瘤2例.脉络膜肿瘤活检标本与肿瘤局部切除标本的病理检查结果具有高度一致性(Kappa=1,P=0.02).结论 玻璃体和脉络膜肿瘤活检标本与肿瘤局部切除标本的病理诊断结果具有较好的一致性.眼内活检技术可作为临床诊断脉络膜肿瘤的可靠手段之一.(中华眼科杂志,2011,47:487-491)

Abstract：

Objective To observe the clinical results of transvitreal choroidal biopsies in the diagnosis of choroidal tumors. Methods It was a retrospective case series study. Slitlamp, indirect ophthalmoscopy, B scan, CDI, MRI, OCT, FFA and ICGA were performed in all 16 cases. The choroidal biopsy was obtained via an internal approach with a three-port pars plana vitrectomy. Vitreous samples were obtained in patients with vitreous hemorrhage. In choroidal tumor located at or in front of the equator, local excision of tumor were performed. The vitreous specimens, choroidal biopsies and excised tumor specimens were analyzed by the histopathologic studies. Statistical analysis was performed using a commercially available statistical software package (SPSS for Windows, version 13.0, SPSS, Chicago, IL). Results In 4 cases of vitreous hemorrhage, histopathologic studies showed hemorrhage in 2 cases and choroidal melanoma cells in another two cases. In 16 choroidal biopsies, 2 cases had a negative results, 11 cases showed choroidal melanoma, 1 case was melanocytoma and 2 cases were hemorrhage only. The diagnostic rate was 87.5%. In 12 cases that underwent local excision, 9 cases were choroidal melanoma, 1 case was melanocytoma and 2 cases were Schwannoma. High concordance was present between choroidal biopsy and local excision(Kappa=1,P=0.02). Conclusions The results obtained from transvitreal choroidal biopsies were consistent with that from local excision. This procedure can be used in the diagnosis and treatment of choroidal tumors.     

A case of nodular posterior scleritis mimicking choroidal mass

The aim of this study is to report clinical and imaging findings, and treatment outcomes of a patient with nodular posterior scleritis. A 41-year-old woman was diagnosed as nodular posterior scleritis in the light of clinical and imaging findings. At first admission best corrected visual acuity was 20/50 in her right eye. Fundus examination revealed an amelanotic subretinal mass under the superior temporal arcade associated with subretinal fluid surrounding it. B-scan ultrasonography, optical coherence tomography, fluorescein angiography, and indocyanine green angiography findings confirmed the diagnosis. As treatment, nepafenac eye drops 3 times a day, and flurbiprofen tablet 100 mg twice a day were prescribed. After 4 weeks of treatment, the ocular pain was relieved, BCVA improved to 20/20, and subretinal mass totally regressed. Although the diagnosis of nodular posterior scleritis may be confusing, it has to be kept in mind in patients with a subretinal/choroidal mass. Multimodal fundus imaging may be helpful in differential diagnosis. The condition is usually curable with non-steroidal anti-inflammatory drugs and/or systemic steroids.