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[年轻患者的葡萄膜和虹膜睫状体黑色素瘤:57例回顾性分析]

[Uveal and iridociliary melanomas in young patients : A retrospective analysis of 57 cases].

作者信息

Heyer L J, Metz C, Flühs D, Heyer C M, Bornfeld N

机构信息

Klinik für Erkrankungen des hinteren Augenabschnittes, Universitätsklinikum Essen, Essen, Deutschland.

Medizinische Physik der Strahlenklinik, Universitätsklinikum Essen, Essen, Deutschland.

出版信息

Ophthalmologe. 2016 Dec;113(12):1046-1050. doi: 10.1007/s00347-016-0285-x.

Abstract

BACKGROUND

Uveal melanomas (UM) are rare malignancies in young patients. It is unknown if UM in young patients significantly differs from those in older patients concerning tumor size and localization, metastasis and genetics. The aim of this study was to evaluate the clinical course and tumor characteristics in young patients with UM.

MATERIAL AND METHODS

All patients with UM below the age of 32 years who had been treated at our hospital were included in the study. Patient age and sex, duration of symptoms, visual impairment, tumor size and location, genetics, therapy, follow-up interventions and tumor-associated deaths were documented.

RESULTS

A total of 57 patients (67 % male, mean age 24.7 years) were included in the study with an average symptomatic course of 5 months. Of the patients 8 (14 %) had an initial visual acuity of ≥ 0.9, 16 (28 %) 0.5-0.8, 22 (39 %) 0.05-0.4 and 9 (16 %) < 0.05 (no data for 2 patients, 4 %). After therapy visual acuity was < 0.05 in 54 % and 53 % of the tumors were choroidal UM (70 % juxtapapillary/circumpapillary), whereas 47 % were ciliochoroidal (54 % with iridociliary involvement). The average tumor size was 12.7 ± 3.6 mm with an average prominence of 6.2 ± 3.2 mm. Genetic evaluation (n = 16) revealed disomy 3 in 64 % and 54 % of the patients received radiotherapy with local application of ruthenium 106. In 46 % of cases follow-up interventions were neccessary including 70 % due to radiogenic retinopathy.

CONCLUSION

In young patients UM did not show any preferred localization. The majority of genetically evaluated tumors revealed disomy 3 with no significant correlation to tumor location. Independent of tumor size, location and therapy, approximately half of the patients needed follow-up interventions, predominantly due to radiogenic retinopathy.

摘要

背景

葡萄膜黑色素瘤(UM)在年轻患者中是罕见的恶性肿瘤。年轻患者的UM在肿瘤大小、定位、转移和遗传学方面是否与老年患者有显著差异尚不清楚。本研究的目的是评估年轻UM患者的临床病程和肿瘤特征。

材料与方法

本研究纳入了所有在我院接受治疗的32岁以下UM患者。记录患者的年龄、性别、症状持续时间、视力损害、肿瘤大小和位置、遗传学、治疗、随访干预措施以及与肿瘤相关的死亡情况。

结果

本研究共纳入57例患者(67%为男性,平均年龄24.7岁),平均症状持续时间为5个月。患者中,8例(14%)初始视力≥0.9,16例(28%)为0.5 - 0.8,22例(39%)为0.05 - 0.4,9例(16%)<0.05(2例患者无数据,占4%)。治疗后,54%的患者视力<0.05,53%的肿瘤为脉络膜UM(70%为邻乳头/乳头周围型),而47%为睫状体脉络膜UM(54%伴有虹膜睫状体受累)。平均肿瘤大小为12.7±3.6mm,平均隆起度为6.2±3.2mm。基因评估(n = 16)显示64%的患者存在3号染色体二体性,54%的患者接受了局部应用钌106的放射治疗。46%的病例需要进行随访干预,其中70%是由于放射性视网膜病变。

结论

在年轻患者中,UM没有表现出任何特定的定位偏好。大多数经过基因评估的肿瘤显示3号染色体二体性,与肿瘤位置无显著相关性。无论肿瘤大小、位置和治疗情况如何,约一半的患者需要进行随访干预,主要原因是放射性视网膜病变。

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