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新西兰/奥特亚罗瓦的虹膜黑色素细胞肿瘤:在高紫外线暴露环境下的表现、治疗和结局。

Iris melanocytic tumours in New Zealand/Aotearoa: presentation, management and outcome in a high UV exposure environment.

机构信息

Department of Ophthalmology, New Zealand National Eye Centre, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand.

Department of Ophthalmology, Greenlane Clinical Centre, Auckland District Health Board, Auckland, New Zealand.

出版信息

Eye (Lond). 2023 Mar;37(4):692-699. doi: 10.1038/s41433-022-02017-2. Epub 2022 Mar 25.

DOI:10.1038/s41433-022-02017-2
PMID:35338357
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9998588/
Abstract

BACKGROUND/OBJECTIVES: Iris melanoma, a rare intraocular malignancy, represents the smallest subgroup of uveal melanoma. This first, comprehensive study of iris melanocytic lesions in the high ultraviolet environment in New Zealand/ Aotearoa (NZ) examines diagnosis, management and outcomes.

SUBJECTS/METHODS: Retrospective study of iris melanocytic tumours referred to tertiary referral centres in Auckland, NZ, over 20 years (1999-2018). Data analysed include demographics, tumour characteristics, histology, genetic analyses, treatment modalities, recurrence, metastasis, 5-year and overall survival.

RESULTS

Cohort (N = 51) was predominantly NZ European (98.0%) with no indigenous Māori, or Pasifika. Median age at presentation was 58 years. Tumours involved a median of two clock hours of iris. The posterior tumour margin extended to the anterior chamber angle in 22 patients (45.8%). Management included initial observation 54.9%, iridectomy/excision biopsy 29.4%, irido-cyclectomy 7.8%, plaque radiotherapy 7.8%, proton beam radiotherapy 7.8%, and ultimately enucleation 17.6%. Histology was performed in 19 cases (37%) with 16 confirmed melanomas (84%). Mean follow-up 4.2 years with median visual acuity of 6/7.5 two years post intervention. Melanoma-related metastasis and mortality occurred in two cases with five-year melanoma-related mortality of 2.0%.

CONCLUSION

In a climate with high ultraviolet exposure iris melanocytic tumours occurred almost exclusively in NZ Europeans, however, the majority of cases were category T1, possibly reflecting early diagnosis in the NZ health system. Nonetheless, >50% underwent surgery or radiotherapy, often utilising more than one modality. A high index of suspicion and early referral of iris melanocytic lesions should be considered in regions with high UV exposure.

摘要

背景/目的:虹膜黑色素瘤是一种罕见的眼内恶性肿瘤,是葡萄膜黑色素瘤中最小的亚群。这是在新西兰/奥特亚罗瓦(NZ)高紫外线环境下对虹膜黑色素细胞病变进行的首次全面研究,旨在检查诊断、治疗和结果。

受试者/方法:对 20 年来(1999-2018 年)转诊至奥克兰三级转诊中心的虹膜黑色素瘤肿瘤患者进行回顾性研究。分析的数据包括人口统计学、肿瘤特征、组织学、遗传分析、治疗方式、复发、转移、5 年和总生存率。

结果

队列(N=51)主要为新西兰欧洲人(98.0%),没有毛利人或太平洋岛民。中位发病年龄为 58 岁。肿瘤累及虹膜中位数为 2 个时钟小时。22 例(45.8%)患者的肿瘤后缘延伸至前房角。治疗方法包括初始观察(54.9%)、虹膜切除术/活检(29.4%)、虹膜切除术/切除术(7.8%)、贴剂放疗(7.8%)、质子束放疗(7.8%)和最终眼球摘除术(17.6%)。19 例(37%)进行了组织学检查,其中 16 例证实为黑色素瘤(84%)。中位随访时间为 4.2 年,干预后 2 年的中位视力为 6/7.5。2 例发生与黑色素瘤相关的转移和死亡,5 年黑色素瘤相关死亡率为 2.0%。

结论

在紫外线暴露高的环境中,虹膜黑色素细胞肿瘤几乎仅发生在新西兰欧洲人中,然而,大多数病例为 T1 类别,可能反映了新西兰卫生系统的早期诊断。尽管如此,>50%的患者接受了手术或放疗,通常采用多种治疗方式。在紫外线暴露高的地区,应高度怀疑并早期转诊虹膜黑色素细胞病变。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e36/9998588/a0427bbb7136/41433_2022_2017_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e36/9998588/a0427bbb7136/41433_2022_2017_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e36/9998588/a0427bbb7136/41433_2022_2017_Fig1_HTML.jpg

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Chromosome 3 and 8q Aberrations in Uveal Melanoma Show Greater Impact on Survival in Patients with Light Iris versus Dark Iris Color.葡萄膜黑色素瘤中3号染色体和8q畸变对浅虹膜与深虹膜颜色患者生存的影响更大。
Ophthalmology. 2022 Apr;129(4):421-430. doi: 10.1016/j.ophtha.2021.11.011. Epub 2021 Nov 13.
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Genetic drivers of non-cutaneous melanomas: Challenges and opportunities in a heterogeneous landscape.
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Presence and prevalence of UV related genetic mutations in uveal melanoma: similarities with cutaneous melanoma.葡萄膜黑色素瘤中与 UV 相关的基因突变的存在和流行:与皮肤黑色素瘤的相似性。
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