儿童眼部肿瘤特点(附119例临床分析)

目的　分析儿童眼部肿瘤的发病特点。方法　对 1 1 9例儿童眼部肿瘤患者的年龄、性别、发病年龄、眼别、肿瘤发生部位及病理分型进行回顾性分析。结果　患儿平均发病年龄为 3 5岁。1 1 9例儿童眼部肿瘤中 ,良性肿瘤 1 3种 53例 ( 44 5% ) ,恶性肿瘤 3种 66例 ( 55 5% )。结论　视网膜母细胞瘤 63例 ( 52 9% ) ,在恶性肿瘤中发病率最高。从发现症状到住院治疗间隔时间 ,视网膜母细胞瘤眼内期为 2 7月 ,青光眼期 5 3月 ,眼外期 1 3 7月 ,视网膜母细胞瘤不同时期的平均发生时间有显著性差异 (P <0 0 5)。嗅神经母细胞瘤的发病率在恶性肿瘤中居第二位。嗅神经母细胞瘤对放疗敏感 ,但易复发     

急性视网膜环死(ARN)—桐泽型葡萄膜炎

浦山晃(Urayama)等于1971年报告6例急性葡萄膜炎,临床表现为周边部渗出性葡萄膜炎、视网膜动脉周围炎和视网膜脱离,命名为桐泽型(Kirisawa type)葡萄膜炎。作者强调此病有11个特点,即:(1)单眼性;(2)急剧发作的泛发性葡萄膜炎;(3)视网膜动脉多灶性浸润;(4)周边眼底有浓密的渗出斑;(5)玻璃体混浊严重;(6)发病后2～3个月内视网膜萎缩,形成多数裂孔及视网膜脱离;(7)视力预后不良;(8)不反复发作;(9)全身无异常     

B型超声波在视网膜母细胞瘤诊断中的应用

视网膜母细胞瘤是婴幼儿最常见的一种眼内恶性肿瘤 ,对视力和生命有严重的威胁和危害 [1 ]。我们自 1993年 10月～ 2 0 0 0年 3月 ,对 39例 48眼视网膜母细胞瘤的患儿 ,术前常规进行眼球 B型超声波探查 ,并与手术及病理结果相对照。现报告如下。1　资料和方法1.1　一般资料　视网膜母细胞瘤患儿 39例 48眼 ,男 2 3例2 9眼 ,女 16例 19眼 ,年龄 5 mo～ 6 a,平均 2 .6 a,病程 1mo～1a,平均 7mo,单眼发病 30例 ,双眼发病 9例。1.2　仪器和方法　用国产眼科 A/ B型超声诊断仪 ,探头频率为 10 MHz,扫描方式 :机械扇形。患儿取平卧位闭眼 ,用直…     

浸润性视网膜母细胞瘤一例

视网膜母细胞瘤以其特有的发病年龄,瞳孔白光反射(猫眼),眼底镜检查见到肿块,B超、CT检查提示实质性肿块及瘤内钙化等特点,一般不难诊断.但如缺乏上述特征,如患儿发病年龄大,眼内无肿块,B超与CT检查无钙化,此时诊断较为困难.弥漫性浸润性视网膜母细胞瘤凶瘤细胞沿着视网膜呈浸润性生长而不形成肿块,绝大多数患者也无钙化,使诊断难度加大.我们曾于2005年收治1例浸润性视网膜母细胞瘤患儿,现回顾分析其早期诊断问题,以总结其经验与教训.     

幼儿团球状脉络膜结核误诊为视网膜母细胞瘤

团球状脉络膜结核,为眼科的少见疾病。发生在幼儿,国内尚未见报导。我院1979年曾遇1例,因临床诊断为视网膜母细胞瘤而行眼球摘除,术后经病理检查确诊为团球状脉络膜结核,随诊6年多无特殊,特作报导。患儿孙××女1岁山东蓬莱人住院号S2241。其父母发现患儿左眼发红,继之视力减退月余。于79年9月初来我院门诊,初诊为视网膜母细胞瘤,后去青岛     

双眼视网膜母细胞瘤1例

1临床资料患者,男,8岁,因左眼视物障碍4月余入院。4月前无任何诱因出现左眼视力下降及眼红,无疼痛,未诊疗;后因左眼视物不见,于兰州大学第二医院就诊,诊断为“左眼视网膜母细胞瘤?”。今来我院门诊检查后,以“(1)左眼Coat’s病;(2)左眼视网膜母细胞瘤(RB)?”收入院。视力:右0.8,左无光感;双眼睑无畸形,球结膜无充血,角膜透明。左眼虹膜表面见新生血管,晶状体后囊轻度混浊,玻璃体腔内灰白色混浊间杂黄色反光,眼底窥视不见;右眼屈光间质清,眼底:视盘边界清,鼻上象限可见近扇形灰白色隆起,约3PD大小,距视盘1.5PD,边界清,病灶内见灰白色钙化…     

低眼压性视网膜母细胞瘤2例

视网膜母细胞瘤(retinoblastoma,RB)是婴幼儿较常见的眼部恶性肿瘤,早期往往不引起家长注意,至青光眼期方来就诊。我们遇到2例低眼压性视网膜母细胞瘤患儿,报告如下。1　临床资料例1　患者,男,1a。主诉左眼红、眼球变小10d。家长否认发病前有发热及外伤史,家族中无同类病?..     

与视网膜母细胞瘤预后有关因素的临床探讨

视网膜母细胞瘤(简称Rb)是一种起源于视网膜核层原始细胞的恶性肿瘤,具有家族和遗传特性。多数发生于婴幼儿,2/3发生于3岁以下的儿童,是婴儿眼内最常见的恶性肿瘤之一,发病率为1:16,000～34,000。其恶性程度高,但若能正确早期诊断,早期治疗,尚可挽回部分患儿的生命,并可保存部分视力。现将我院近10年来收治的16例Rb病人的治疗及预后情况报告如下:     

244例儿童视网膜母细胞瘤临床特点

目的 分析和探讨儿童视网膜母细胞瘤(Rb)的临床特点.设计 回顾性病例系列.研究对象 北京同仁医院2009年1月至12月收治的244例(312眼)儿童视网膜母细胞瘤患者的病历资料.方法 对上述患儿临床病历资料进行回顾,分析患儿的发病年龄、首诊症状、家族史,肿瘤的临床分期、治疗方法、随诊结果及疗效等.主要指标 发病年龄、...     

Coats病临床病理学观察(附七例报告)

1.本文报告七例幼年Coats 病。临床表现有累及单眼、男性倾向、视力下降和瞳孔区有黄光反射。眼底镜检查可见视网膜脱离和视网膜下渗出。病理检查符合视网膜毛细血管扩张和微动脉瘤形成、渗出性视网膜全脱离。2.文献广泛讨论了以血管学说为主的发病机理,但迄今尚未最后确定病因因素。因此目前甚难有相应预防措施。作者等推测Coats 病象急性肾炎样、以全身过敏性感染性炎症为始动因素,主要累及视网膜血管病变。3.建议临床开展萤光血管造影检查,注意鉴别视网膜母细胞瘤和早期治疗。     

双眼异时性视网膜母细胞瘤21例临床分析

目的　通过分析双眼异时性视网膜母细胞瘤（RB）患儿的临床资料,确定高危因素,制订合理的随访计划,从而使肿瘤得以早期发现、早期治疗。方法　回顾性病例分析。收集2014年12月至2019年3月北京儿童医院收治的第一眼确诊为RB后,第二眼发现新发RB的病例共21例,分析双眼确诊间隔时间、发病年龄、首发症状,以及第二眼的确诊方法与肿瘤分期（IIRC）关系等。眼底检查以全身麻醉下间接眼底镜联合RetCam作为常规检查。结果　第一眼发病时患儿的年龄为1~36个月,平均12.2个月,13例年龄≤12个月;第二眼发病年龄为3~84个月,平均25.7个月,19例年龄≤36个月;双眼发病间隔时间为1~48个月,平均13.7个月。第二眼有首发症状者4例,17例（81.0%）在随诊例行检查时发现,无临床症状。第二眼肿瘤分期:A期8例,B期4例,C期1例,D期6例,E期2例。第二眼肿瘤以全身麻醉下间接眼底镜联合RetCam检查发现为主,患者多为早期。结论　第一眼RB发病年龄≤12个月是第二眼发生RB的高危因素,双眼确诊的间隔时间多≤ 36个月;定期、规律随诊和全身麻醉下间接眼底镜联合RetCam检查是早期发现第二眼RB的有效方法。     

Lateralization phenomenon in retinoblastoma patients

Clinical and histopathological findings in a group of 28 infants suffering from retinoblastoma were analyzed. The authors were able to demonstrate that the retinoblastomas occurred considerably more often in the left eye than in the right. In children whose average age was under 2 years tumors found in the left eye were more differentiated. The authors point out that nephroblastomas (Wilm's tumor), like retinoblastomas, occur more frequently in the left eye and have a better prognosis in patients under 2 years of age.     

Malignant tumours of the eye and adnexa

We analysed 262 consecutive histopathologically proved cases of malignant tumours of the eye and its adnexa seen over a nine year period. Two peaks were observed in the age distribution one in the first decade due to retinoblastoma and another between forty one to sixty years due to malignant lid tumours especially meibomian carcinoma. Males (56%) were affected more often. Intraocular tumours (34%) formed the commonest group of malignant orbito-ocular tumours; lacrimal gland tumours (3%) occurred least frequently. Of the individual tumours, retinoblastoma (32%) was seen most often followed by squamous cell carcinoma (25%). The frequency of meibomian gland carcinoma in Madras was significantly more than in North India. Extra-ocular malignant melanomas out-numbered intraocular (uveal) malignant melanomas. The significance of these observations is discussed.     

Increased aqueous lactic dehydrogenase in Coat's disease

A case of early childhood Coats's disease in a ten-month-old boy is presented. The lactic dehydrogenase aqueous:serum ratio was very high (6:1). A ratio higher than 3:1 in Coats's disease or in any other eye infection has never been reported except in cases of retinoblastoma. Since retinoblastoma does not always show calcification in x-rays, it may be concluded that in certain cases it would be impossible to discriminate between Coats's disease and retinoblastoma (except in pathologic analysis) with any available diagnostic tools.     

The effect of initial recognition of abnormalities by physicians on outcome of retinoblastoma.

PURPOSE: The purpose of this study was to assess the effect of early diagnosis by physicians on outcome of retinoblastoma. METHODS: This was an observational case series in which the records of 36 patients with retinoblastoma were reviewed. The age at onset of first symptoms and diagnosis, identity of the individual who first recognized an abnormality, treatment, and outcome were analyzed. RESULTS: Twenty-two patients had unilateral retinoblastoma. Both eyes were preserved in only 2 of these patients, both of whom had abnormalities first noted by their pediatricians. Fourteen patients had bilateral retinoblastoma. Both eyes were preserved in the 5 bilateral patients who were monitored from birth with serial examinations under anesthesia because of family histories of retinoblastoma. Enucleation of at least 1 eye was required in 8 of the 9 bilateral patients whose families first noted abnormalities. Enucleation of at least 1 eye was required in 27 of 28 patients (96%) in whom abnormalities were first noted by parents. CONCLUSION: In this series, the only patients with unilateral retinoblastoma who did not require enucleation were those in whom pediatricians, rather than family members, first noted ocular abnormalities. Both eyes were preserved in all patients with bilateral retinoblastoma who underwent serial examinations under anesthesia because of family histories of retinoblastoma. The prognosis for retinoblastoma appears to be greatly improved when physicians initially detect abnormalities. Educational efforts directed at early detection of retinoblastoma by pediatricians and other primary care providers should be encouraged.     

Prognostic significance of aqueous humour lactic dehydrogenase activity.

The lactic dehydrogenase (LDH) activity of the aqueous humour has been estimated in both eyes of 7 patients having uniocular retinoblastoma. In 1 patient the aqueous humour LDH activity in the healthy eye was above normal, but there was no clinical evidence of malignancy. Tumour tissue was detected in this eye 9 months later, and the aqueous humour showed a rise in LDH activity. A high LDH activity persisted even after irradiation, though no tumour tissue was visible ophthalmoscopically. It is suggested that the estimation of the LDH activity in the aqueous humour of the healthy eye in cases of uniocular retinoblastoma might be of value in the early detection of a bilateral retinoblastoma.     

Diffuse Anterior Retinoblastoma with Sarcoidosis-Like Nodule

Background

Retinoblastomas account for 4% of malignancies in children, 1-2% of which are diffuse infiltrating retinoblastomas. Diffuse anterior retinoblastoma is rare and does not involve the retina. Here, we report on a diffuse anterior retinoblastoma with large sarcoidosis-like nodules on the iris that were responsive to anti-inflammatory therapy.

Case

We present a 6-year-old girl who had anterior uveitis with white nodules on the iris and posterior surface of the cornea in her right eye. The nodules initially responded well to anti-inflammatory treatment. However, anterior segment optical coherence tomography (AS-OCT) showed that the nodules gradually grew, shrinking the iris. We then collected the aqueous humor for diagnosis. A biopsy revealed clusters of small cells with a high nuclear-to-cytoplasm ratio with partial rosette formation. Therefore, we diagnosed diffuse anterior retinoblastoma without retinal involvement and performed enucleation of the right eye. The histopathology demonstrated undifferentiated cells similar to those seen on the biopsy, and tumor cells invaded the iris stroma, posterior surface of the cornea, ciliary body, and sclera. After the enucleation, she underwent chemotherapy and remains alive.

Conclusion

A differential diagnosis of retinoblastoma should be considered when white nodules refractory to anti-inflammatory therapy occur in the eye, even in the absence of obvious retinal masses. AS-OCT findings are useful in assessing retinoblastoma.Key Words: Retinoblastoma, Diffuse anterior retinoblastoma, Sarcoidosis, Diffuse infiltrating retinoblastoma, Enucleation, Chemotherapy, Anterior segment optical coherence tomography     

Experience in treatment of retinoblastoma in the German Democratic Republic (author's transl)

A report on 150 patients suffering from retinoblastoma (71 unilateral and 79 bilateral cases) treated between 1960 and 1977. There was a familiar accumulation of retinoblastoma in 8.9% of the bilateral and 5.8% of the unilateral cases. Leukokoria and squint were the most frequent first signs of the tumor. The overall lethality was 12.7% during an average follow up period of 7.8 years. The lethality due to metastases on bilateral cases (8.9%) was as high as that due to unilateral retinoblastomas (9.9%). Spontaneous cure occurred in 2%. The cure rate of unilateral retinoblastomas was 87.3%. The survival rate in bilateral tumor cases 84.8%. The eye with the more advanced tumor was enucleated immediately; irradiation therapy only saved both eyes in one patient. Enucleation of the second eye was necessary in 40.5% of our patients suffering from bilateral retinoblastoma. In 43.8% of all bilateral retinoblastomas destruction of the tumor in the second eye was accomplished and a useful degree of visual acuity was achieved after an average follow-up period of 7.5 years.--The optimum therapeutic procedure depends on the size and position of the retinoblastoma and should be scheduled individually in each patient: photocoagulation, cryocoagulation, 106Ru/ 106Rh-applicator, 60Co-applicators, external beam irradiation (linear accelerator 5 MeV), chemotherapy.     

The clinical outcomes of proton beam radiation therapy for retinoblastomas that were resistant to chemotherapy and focal treatment

Purpose

To evaluate the clinical results of proton beam radiation therapy (PBRT) for treatment of retinoblastoma.

Methods

Children with retinoblastoma who were treated with chemotherapy and focal treatment such as brachytherapy and thermotherapy but showed no response or developed recurrences later received PBRT. The PBRT strategy was designed to concentrate the radiation energy to the retinoblastoma and spare the surrounding healthy tissue or organs.

Results

There were three patients who received PBRT. The first patient received PBRT because of an initial lack of tumor regression with chemotherapy and brachytherapy. This patient showed regression after PBRT. The second patient who developed recurrence of retinoblastoma as diffuse infiltrating subretinal seeding was taken PBRT. After complete regression, there was recurrence of tumor and the eye was enucleated. The third patient had unilateral extensively advanced retinoblastoma. Initial chemotherapy failed and tumor recurred. The tumor responded to PBRT and regressed significantly. However, the eye developed sudden multiple recurrences, so we had to perform enucleation.

Conclusions

PBRT for retinoblastoma was effective in cases of showing no response to other treatment modalities. However, it should be carefully applied when there was recurrence of diffuse infiltrating subretinal seeding or extensively advanced retinoblastoma initially.     

Brachytherapy of intra ocular tumors using ‘BARC I-125 Ocu-Prosta seeds’: An Indian experience

Aim:

To report our experience of brachytherapy using ‘BARC I-125 Ocu-Prosta seeds’ for the management of intraocular tumors with regard to tumor control, globe preservation visual outcome, and patient survival at Sankara Nethralaya, Chennai, India between September 2003 and May 2011.

Materials and Methods:

We reviewed records of 35 eyes of 35 patients who underwent ophthalmic brachytherapy between September 2003 and May 2011. Twenty-one cases had choroidal melanoma, nine had childhood retinoblastoma, two had adult-onset retinoblastoma, and there were one case each of vasoproliferative tumor, retinal angioma, and ciliary body melanoma. Brachytherapy was administered using a 15- or 20-mm gold plaque with or without a notch. Brachytherapy was the primary treatment modality in all tumors other than retinoblastoma, wherein brachytherapy was done post chemoreduction for residual tumor.

Results:

For choroidal melanomas, the mean radiation dose was 68.69 ± 15.07 (range, 47.72-94.2) Gy. The eye salvage rate was 13/20 (65%) and tumor control rate was 16/20 (80%) at an average follow-up of 24.43 ± 24.75 (range, 1.5-87.98) months. For retinoblastoma, the mean dose was 45.85 ± 3.90 (range, 39.51-50.92) Gy. The eye salvage rate and tumor control rate was 5/6 (83.3%) at an average follow-up of 38.36 ± 31.33 (range, 4.14-97.78) months. All eyes with retinoblastoma needed additional focal therapy for tumor control and eye salvage.

Conclusion:

The results of this retrospective study confirms that the use of ‘BARC I-125 Ocu-Prosta seeds’ in episcleral plaques to treat intraocular tumors offers a viable option for the management of intraocular cancers.