PDF(Pubmed)
Abstract：
The purpose of this study was to investigate the clinical features, tumor characteristics, including histopathology and cytogenetic analysis, and management of patients with uveal ring melanoma in New Zealand.
A retrospective review was conducted on all uveal melanoma cases treated in a single national oncology center in New Zealand. The study period was from January 1, 2013 to December 31, 2022 (10 years). Written consent was obtained from all patients included in this case series.
Uveal ring melanoma of ciliary body origin (n = 4) comprised 0.7% of all uveal melanomas (n = 571). Ethnicity distribution was three patients of New Zealand European ancestry and one patient of Chinese/Pasifika ancestry. Three patients (75%) were symptomatic at presentation (spontaneous hyphema, glaucoma, and cataract), whereas one was asymptomatic but subsequently developed painful refractory glaucoma. All eyes underwent enucleation. Three eyes had primary iris biopsies with subsequent enucleation for refractory glaucoma and pain and one eye underwent primary enucleation. All cases demonstrated malignant tumor characteristics including diffuse 270 to 360-degree ciliary body ring growth pattern, epithelioid-cell type and presence of either BAP-1 expression loss or gain of MYC gene. Two cases (50%) developed distant organ metastasis - liver, parotid gland, and breast. Of those, one patient was deceased at the time of follow-up whereas one had completed treatment for metastases.
Uveal ring melanoma is a rare tumor that is highly invasive and malignant. When detected, prompt definitive treatment should be advocated, and patient counselling should be given regarding the high risk of developing a painful, blind eye with increased risk of metastasis in the absence of treatment.
A retrospective review was conducted on all uveal melanoma cases treated in a single national oncology center in New Zealand. The study period was from January 1, 2013 to December 31, 2022 (10 years). Written consent was obtained from all patients included in this case series.
Uveal ring melanoma of ciliary body origin (n = 4) comprised 0.7% of all uveal melanomas (n = 571). Ethnicity distribution was three patients of New Zealand European ancestry and one patient of Chinese/Pasifika ancestry. Three patients (75%) were symptomatic at presentation (spontaneous hyphema, glaucoma, and cataract), whereas one was asymptomatic but subsequently developed painful refractory glaucoma. All eyes underwent enucleation. Three eyes had primary iris biopsies with subsequent enucleation for refractory glaucoma and pain and one eye underwent primary enucleation. All cases demonstrated malignant tumor characteristics including diffuse 270 to 360-degree ciliary body ring growth pattern, epithelioid-cell type and presence of either BAP-1 expression loss or gain of MYC gene. Two cases (50%) developed distant organ metastasis - liver, parotid gland, and breast. Of those, one patient was deceased at the time of follow-up whereas one had completed treatment for metastases.
Uveal ring melanoma is a rare tumor that is highly invasive and malignant. When detected, prompt definitive treatment should be advocated, and patient counselling should be given regarding the high risk of developing a painful, blind eye with increased risk of metastasis in the absence of treatment.
摘要：
■这项研究的目的是调查临床特征，肿瘤特征，包括组织病理学和细胞遗传学分析，新西兰葡萄膜环形黑色素瘤患者的治疗。
■对在新西兰一个国家肿瘤中心治疗的所有葡萄膜黑色素瘤病例进行了回顾性研究。研究期为2013年1月1日至2022年12月31日（10年）。从本病例系列中包括的所有患者获得书面同意。
■睫状体来源的葡萄膜环形黑色素瘤（n=4）占所有葡萄膜黑色素瘤（n=571）的0.7％。种族分布是3名新西兰欧洲血统患者和1名中国/Pasifika血统患者。3例患者（75％）出现症状（自发性前房积血，青光眼,和白内障），而其中一人无症状，但随后发展为疼痛性难治性青光眼。所有眼睛都进行了摘除。三只眼睛进行了初次虹膜活检，随后进行了难治性青光眼和疼痛的摘除，一只眼睛进行了初次摘除。所有病例均表现出恶性肿瘤特征，包括弥漫性270至360度睫状体环生长模式，上皮样细胞类型和BAP-1表达缺失或MYC基因获得的存在。2例（50％）发生远处器官转移-肝脏，腮腺,和乳房。其中,1例患者在随访时死亡,1例患者已完成转移治疗.
■葡萄膜环黑色素瘤是一种罕见的肿瘤，具有高度侵袭性和恶性。检测到时,应该提倡迅速的明确治疗，和病人的咨询应该给予关于高风险的发展痛苦，在没有治疗的情况下，盲眼转移的风险增加。
■对在新西兰一个国家肿瘤中心治疗的所有葡萄膜黑色素瘤病例进行了回顾性研究。研究期为2013年1月1日至2022年12月31日（10年）。从本病例系列中包括的所有患者获得书面同意。
■睫状体来源的葡萄膜环形黑色素瘤（n=4）占所有葡萄膜黑色素瘤（n=571）的0.7％。种族分布是3名新西兰欧洲血统患者和1名中国/Pasifika血统患者。3例患者（75％）出现症状（自发性前房积血，青光眼,和白内障），而其中一人无症状，但随后发展为疼痛性难治性青光眼。所有眼睛都进行了摘除。三只眼睛进行了初次虹膜活检，随后进行了难治性青光眼和疼痛的摘除，一只眼睛进行了初次摘除。所有病例均表现出恶性肿瘤特征，包括弥漫性270至360度睫状体环生长模式，上皮样细胞类型和BAP-1表达缺失或MYC基因获得的存在。2例（50％）发生远处器官转移-肝脏，腮腺,和乳房。其中,1例患者在随访时死亡,1例患者已完成转移治疗.
■葡萄膜环黑色素瘤是一种罕见的肿瘤，具有高度侵袭性和恶性。检测到时,应该提倡迅速的明确治疗，和病人的咨询应该给予关于高风险的发展痛苦，在没有治疗的情况下，盲眼转移的风险增加。
