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加纳一家儿科眼科诊所视网膜母细胞瘤的病例报告

Presentation of retinoblastoma at a paediatric eye clinic in ghana.

作者信息

Essuman V, Ntim-Amponsah C T, Akafo S, Renner L, Edusei L

机构信息

Ophthalmology Unit, Department of Surgery, University of Ghana Medical School, PO Box 4236, Accra, Ghana.

出版信息

Ghana Med J. 2010 Mar;44(1):10-5. doi: 10.4314/gmj.v44i1.68850.

Abstract

BACKGROUND

Retinobalstoma, the commonest childhood malignant intraocular tumour, is usually diagnosed early with over 90% survival rate in developed countries. In developing countries, the diagnosis is late resulting in less than 50% survival.

OBJECTIVE

To determine retinoblastoma stages at presentation and patients' outcomes.

DESIGN

Retrospective case series.

METHODS

The clinical and histopathological records of children with retinoblastoma seen from May 2004 to December 2005 were studied. Data was analysed for mode of presentation, laterality, clinical staging using Reese-Ellsworth (R-E) classification, histopathological high risk features (HHRF) for metastasis, and patient outcome.

SETTING

Ophthalmology Unit, Korle-Bu Teaching Hospital, Ghana.

RESULTS

Twenty-three patients presented with retinoblastoma over the period. Males were 12(52.2%). The age range from 1 to 84 months, mean 36.3(±22.15) and median (36 months). Nineteen (82.6%) had unilateral and 4(17.4%) bilateral disease. The common clinical presentations were leukocoria in 20(87.0%), proptosis 8(34.8%), strabismus 5(21.7%) and red eye 5(21.7%). The clinical features were commensurate with R-E stage V in 20(87.0%) patients, 2(8.7%) with orbital recurrence and 1(4.3%) with post-enucleation anophthalmos. HHRFs were present in 9(75%) enucleated eyes with invasion of optic nerve as the commonest site (7/9). The patients were followed up for 1 day to 19 months. Eight abandoned treatment, 2 were discharged for palliative treatment, 2 out of 5 with metastasis died and 6 had no metastases at their last visit. Common sites for metastasis were the bone marrow, brain and orbit.

CONCLUSION

Majority of the patients presented with advanced disease manifesting as leukocoria, proptosis, RE stages V disease and poor outcome.

摘要

背景

视网膜母细胞瘤是儿童最常见的眼内恶性肿瘤,在发达国家通常能早期诊断,生存率超过90%。在发展中国家,诊断往往较晚,导致生存率低于50%。

目的

确定视网膜母细胞瘤就诊时的分期及患者的预后。

设计

回顾性病例系列研究。

方法

研究2004年5月至2005年12月期间视网膜母细胞瘤患儿的临床和组织病理学记录。分析数据包括就诊方式、患侧性、使用里斯-埃尔斯沃思(R-E)分类法的临床分期、转移的组织病理学高危特征(HHRF)以及患者预后。

地点

加纳科勒-布教学医院眼科。

结果

在此期间有23例患者被诊断为视网膜母细胞瘤。男性12例(52.2%)。年龄范围为1至84个月,平均36.3(±22.15)个月,中位数为36个月。19例(82.6%)为单侧患病,4例(17.4%)为双侧患病。常见的临床表现为白瞳症20例(87.0%)、眼球突出8例(34.8%)、斜视5例(21.7%)和眼红5例(21.7%)。20例(87.0%)患者的临床特征符合R-E分期V期,2例(8.7%)有眼眶复发,1例(4.3%)有眼球摘除术后无眼球。9例(75%)摘除眼球存在HHRF,最常见的部位是视神经侵犯(7/9)。对患者进行了1天至19个月的随访。8例放弃治疗,2例出院接受姑息治疗,5例有转移的患者中有2例死亡,6例在最后一次随访时无转移。转移的常见部位是骨髓、脑和眼眶。

结论

大多数患者就诊时病情已进展,表现为白瞳症、眼球突出、R-E分期V期疾病且预后较差。

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